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diff --git a/docs_md/articles/aberrant-right-subclavian-artery_a20702fe-5409-44a5-a804-cb071023bade.md b/docs_md/articles/aberrant-right-subclavian-artery_a20702fe-5409-44a5-a804-cb071023bade.md
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+++ b/docs_md/articles/aberrant-right-subclavian-artery_a20702fe-5409-44a5-a804-cb071023bade.md
@@ -0,0 +1,326 @@
+---
+title: "Aberrant Right Subclavian Artery"
+docid: "a20702fe-5409-44a5-a804-cb071023bade"
+authors:
+ - key: "770e1d77-2287-436e-910b-48232afc7842"
+ value: "Prabhakar Rajiah, MBBS, MD, FACR, FRCR, FACC, FAHA, FSCCT"
+ - key: "e915766e-8102-46e4-a33e-c83f8ae12f29"
+ value: "Harold Goerne, MD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Aberrant Right Subclavian Artery"
+ slug: "aberrant-right-subclavian-artery"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "69195035-5819-4996-b27d-b107c62d7c60"
+imageCount: 12
+lastUpdated: "01/28/25"
+pageDescription: "Aberrant Right Subclavian Artery"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Aberrant Right Subclavian Artery"
+pageTitle: "Aberrant Right Subclavian Artery | STATdx"
+enhancedTitle: "Aberrant Right Subclavian Artery"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Aberrant Right Subclavian Artery"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Aberrant right subclavian artery (ARSA) as last branch of left-sided aortic arch, isthmus, or proximal descending aorta
+ - Course: Retroesophageal 80%; intertracheoesophageal 15%; pretracheal 5%
+- ## Imaging
+
+
+ - Barium esophagogram shows persistent esophageal narrowing at level of extrinsic compression with oblique course
+ - CTA or MRA help to understand origin and course of supraaortic trunks
+ - No vascular ring formed by left aortic arch and ARSA
+ - Loose vascular ring if ARSA originates from Kommerell diverticulum
+ - Complete vascular ring if ARSA with circumflex right descending thoracic aorta with right ductus
+- ## Top Differential Diagnoses
+
+
+ - Major aortopulmonary collateral arteries (MAPCAs)
+ - Right arch with aberrant left subclavian artery
+ - Retroesophageal diverticulum
+- ## Pathology
+
+
+ - Regression of right 4th arch between right subclavian and right common carotid arteries, including right ductus arteriosus
+- ## Clinical Issues
+
+
+ - Most common congenital aortic arch abnormality (0.5-2.0%)
+ - 90-95% asymptomatic; incidental imaging finding
+ - Adults: Dysphagia (lusoria), dyspnea, back pain
+ - Infants: Cough, stridor, aspiration pneumonia
+ - Surgery for symptoms, aneurysm, or large diverticulum
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Aberrant right subclavian artery (ARSA)
+- ## Synonyms
+
+
+ - Lusoria artery (arteria lusoria)
+ - Incomplete vascular ring
+- ## Definitions
+
+
+ - Aberrant origin of right subclavian artery as last branch of left-sided aortic arch or proximal descending aorta
+ - Normally RSA originates from brachiocephalic artery
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - ARSA as last branch of left-sided aortic arch or proximal descending aorta
+ - Retroesophageal/retrotracheal course of ARSA
+ - ### Location
+
+
+ - ARSA takeoff from distal aortic arch, isthmus, or proximal descending aorta
+ - Course: Retroesophageal 80%; intertracheoesophageal 15%; pretracheal 5%
+ - ### Size
+
+
+ - Usually normal vessel caliber
+ - Dilated proximal segment in Kommerell diverticulum
+ - Aneurysm may be present
+ - ### Morphology
+
+
+ - No vascular ring formed by left aortic arch and ARSA
+ - Trachea and esophagus are in middle between aortic arch and ARSA
+ - Loose vascular ring if ARSA originates from Kommerell diverticulum
+ - Right ductus/ligamentum arteriosum completes ring
+ - Ductus arising from junction between diverticulum and normal-sized subclavian artery
+ - Seen in 15-30% of ARSA
+ - Complete vascular ring if ARSA with circumflex right descending thoracic aorta and right ductus
+ - Arch itself crosses midline, posterior to esophagus
+ - Right ductus extends from descending aorta to right pulmonary artery, completing vascular ring
+- ## Radiographic Findings
+
+
+ - Chest x-ray may show enlargement of superior mediastinum
+ - Barium esophagogram shows persistent esophageal narrowing at level of extrinsic compression with oblique course
+- ## CT Findings
+
+
+ - ### CTA
+
+
+ - Excellent modality to demonstrate aortic arch configuration and branching pattern
+ - MPR, MIP, and VRT reconstructions help to understand origin and course of supraaortic trunks
+ - Evaluates esophageal and tracheal compression
+ - NECT: May be seen incidentally
+ - 4D CT: May show dynamic esophageal compression during cardiac cycle due to distension of ARSA during systole
+- ## MR Findings
+
+
+ - ### MRA
+
+
+ - Arch vessel branching pattern is (from right to left): Right common carotid artery; left common carotid artery; left subclavian artery; right subclavian artery (RSA)
+ - Course: Obliquely from caudal left to cranial right
+ - Aneurysm of ARSA: 1.5x d of distal subclavian artery
+ - Left ductus may be seen; no vascular ring
+ - Since there is vasculature on only 3 sides of trachea and esophagus
+ - Posterior esophageal compression in 10%
+ - Usually in 4th or 5th decade
+ - Stretching, sclerosis/calcification, aneurysm of ARSA
+ - Complete vascular ring may be seen if
+ - Kommerell diverticulum: Dilation of origin of ARSA
+ - Diameter of orifice of diverticulum and distance from furthest aortic wall to end of diverticulum measured
+ - Circumflex right descending thoracic aorta with arch behind esophagus
+ - ### SSFP cine
+
+
+ - May show dynamic esophageal compression during cardiac cycle due to vessel distension during systole
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - CTA or MRA
+ - ### Protocol advice
+
+
+ - ECG gating is not necessary if only evaluation of vascular ring is desired
+ - High-pitch helical mode of dual source CT scanner (Flash) mode, provide anatomic information
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Major Aortopulmonary Collateral Arteries
+
+
+ - Collateral vessels arising from descending aorta that end in lungs in patients with pulmonary artery atresia
+- [Right Arch With Aberrant Left Subclavian Artery](/document/right-aortic-arch/5f186c96-4cc3-453e-840d-12ebfad13115)
+ - Mirror image of left arch with ARSA
+ - Caused by persistent right 4th arch and regression of left 4th arch in between left common carotid and left subclavian arteries
+ - Aberrant left subclavian artery originates as last branch from right aortic arch and courses behind esophagus to reach left
+ - 90% have left ductus vascular ring
+ - 2nd most common cause of complete vascular ring
+- ## Retroesophageal Diverticulum
+
+
+ - Outpouching at origin of aberrant left subclavian artery in right aortic arch; seen in 60% of these cases
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Embryologic insult during formation of aortic arches
+ - Right 4th arch regresses between RSA and right common carotid artery, including ductus arteriosus
+ - Distal right dorsal aorta forms proximal RSA
+ - Distal portion from 7th right intersegmental artery
+ - Kommerell diverticulum: Remnant of dorsal aortic arch
+ - Persistence of right 6th arch component that forms right ductus arteriosus
+ - Left circumflex aorta
+ - Regression of right 4th arch between right common carotid and right subclavian arteries
+ - Persistence of right 6th arch component that forms ductus
+ - Right-sided descending aorta
+ - Distal left dorsal aorta forms definitive distal aortic arch
+ - ### Associated abnormalities
+
+
+ - ARSA is present in 35% of patients with Down syndrome
+ - Kommerell diverticulum: 14.9%
+ - Aneurysm of distal RSCA: 12.8%
+ - Others: Coarctation; patent ductus arteriosus; ventricular septal defect; truncus bicaroticus; type B interrupted arch
+- ## Staging, Grading, & Classification
+
+
+ - Retroesophageal course: 80%
+ - Interesophageotracheal course: 15%
+ - Pretracheal course: 5%
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - 90-95% asymptomatic; usually incidental finding in imaging studies
+ - ### Other signs/symptoms
+
+
+ - Adults: Dysphagia (lusoria), dyspnea, back pain, arm claudication, abnormal chest x-ray
+ - Infants: Cough, stridor, occasionally aspiration pneumonia
+ - Rare: Ruptured diverticulum; dissection
+- ## Demographics
+
+
+ - Age: Any; mean at detection 41.8 ± 26.6 years
+ - Sex: F:M = 3:1
+ - Epidemiology
+ - Most common congenital aortic arch abnormality
+ - Prevalence 0.5-2.0% of population
+- ## Natural History & Prognosis
+
+
+ - Caution required during esophageal surgeries
+ - Caution required in thyroid and parathyroid surgeries
+ - Right inferior laryngeal nerve passes directly from vagus nerve at level of larynx to neck
+- ## Treatment
+
+
+ - Surgery for symptoms, aneurysm, or large diverticulum
+ - Diverticulum orifice > 3-cm or > 5-cm depth between wall adjacent to trachea and opposite aortic wall
+
+ 6f7d98b5-f858-4f63-8153-640ab0b652fd
+
+## References
+
+# Selected References
+
+1. [Robb CL et al: Subclavian artery: anatomic review and imaging evaluation of abnormalities. Radiographics. 42(7):2149-65, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36053845%5Bpmid%5D)
+1. [Mazzaccaro D et al: Analysis of origin of the supra-aortic trunks from the aortic arch. J Vasc Surg. 67(2):399-408, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28830708%5Bpmid%5D)
+1. [Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27860551%5Bpmid%5D)
+1. [Polednak AP: Prevalence of the aberrant right subclavian artery reported in a published systematic review of cadaveric studies: the impact of an outlier. Clin Anat. 30(8):1024-8, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28514512%5Bpmid%5D)
+1. [Allen D et al: Arteria lusoria: an anomalous finding during right transradial coronary intervention. Case Rep Cardiol. 2016:8079856, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27478652%5Bpmid%5D)
+1. [Tanaka A et al: Kommerell's diverticulum in the current era: a comprehensive review. Gen Thorac Cardiovasc Surg. 63(5):245-59, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25636900%5Bpmid%5D)
+1. [Etesami M et al: Computed tomography in the evaluation of vascular rings and slings. Insights Imaging. 5(4):507-21, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25008430%5Bpmid%5D)
+1. [Mahmodlou R et al: Aberrant right subclavian artery: a life-threatening anomaly that should be considered during esophagectomy. J Surg Tech Case Rep. 6(2):61-3, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25598945%5Bpmid%5D)
+1. [Roofthooft MT et al: Down syndrome and aberrant right subclavian artery. Eur J Pediatr. 167(9):1033-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18172685%5Bpmid%5D)
+1. [Donnelly LF et al: Aberrant subclavian arteries: cross-sectional imaging findings in infants and children referred for evaluation of extrinsic airway compression. AJR 178:1269-74, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11959744%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
, left internal carotid artery 
, left external carotid artery 
, left subclavian artery 
, and aberrant right subclavian artery (ARSA) 
. Mild aortic coarctation 
is also noted.](images/app.statdx.com_image_thumbnail_87b003df-dee1-4d51-890f-1f947af7b590_annotated_true_size_900_quality_90_181333cebaf67cc8ff05e7b3df5c1183cab2cecf.jpg)
+*CTA cinematic rendering reconstruction shows 5 branches from the aortic arch. From the proximal to distal, the branches are: Right common carotid artery , left internal carotid artery , left external carotid artery , left subclavian artery , and aberrant right subclavian artery (ARSA) . Mild aortic coarctation is also noted.*
+
+
+*CTA cinematic rendering reconstruction shows 5 branches from the aortic arch. From the proximal to distal, the branches are: Right common carotid artery , left internal carotid artery , left external carotid artery , left subclavian artery , and aberrant right subclavian artery (ARSA) . Mild aortic coarctation is also noted.*
+
+
+*CTA cinematic rendering reconstruction shows ARSA that originates as the last branch from the aortic arch and courses behind the esophagus to reach the right.*
+
+
+*Coronal oblique MIP CTA shows a prominent Kommerell diverticulum , which is a dilatation of a proximal ARSA that originates in the proximal descending aorta .*
+
+
+*Axial oblique MIP reconstruction from a CTA shows an ARSA with retroesophageal course causing esophageal compression between aortic arch, trachea , and ARSA.*
+
+
+### Additional Images
+
+
+*Axial oblique MIP reconstruction from a CTA shows an ARSA that causes esophageal compression .*
+
+
+*3D reconstruction of the airways and lungs from a CT in the same patient shows severe esophageal luminal narrowing from an ARSA.*
+
+
and right vertebral artery 
, left internal carotid artery , left external carotid artery 
, left vertebral artery , left subclavian artery , and ARSA .](images/app.statdx.com_image_thumbnail_ee1ce812-91ce-4836-a7d8-26c425081e90_annotated_true_size_900_quality_90_5423ed25cea6e5dcb724ee235086d4bb1aa9edb7.jpg)
+*MRA 3D posterior oblique view shows 6 branches from the aortic arch. From proximal to distal the branches are: A common trunk dividing into right common carotid artery and right vertebral artery , left internal carotid artery , left external carotid artery , left vertebral artery , left subclavian artery , and ARSA .*
+
+
+*Sagittal T2-weighted MR of the cervical spine shows a round structure between the spine and esophagus , consistent with an aberrant right subclavian artery causing esophageal compression.*
+
+
+*Coronal MIP reconstruction from a MRA shows an ARSA and a normal left subclavian artery , both originating at the same level in the distal aortic arch .*
+
+
+*CTA cinematic rendering reconstruction shows a right-sided aortic arch with mirror-image branching and aberrant left subclavian artery , which originates as the last branch from the arch.*
+
+
+*Coronal MIP CTA reconstruction in a 56-year-old man shows an ARSA originating from the proximal descending thoracic aorta and extending towards the right.*
+
+
+*Axial MIP CTA reconstruction in a 56-year-old man shows an ARSA originating from the proximal descending thoracic aorta and extending towards the right, with compression of the esophagus .*
+
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@@ -0,0 +1,367 @@
+---
+title: "Aortic Aneurysm: Rupture"
+docid: "e64e6a27-0c8d-4b4f-8ca5-d2e65c9f2e9a"
+authors:
+ - key: "b00d2bdb-66e1-41ed-90b4-c52904f4d598"
+ value: "Seth Kligerman, MD, MS"
+ - key: "9ad9af12-61a1-44d6-af52-0ee1f38eb298"
+ value: "Davis Vigneault, MD, DPhil"
+ - key: "5de0df07-7b3e-4678-8767-1519e1153f29"
+ value: "Dominik Fleischmann, MD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Aortic Aneurysm: Rupture"
+ slug: "aortic-aneurysm-rupture"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "fa15c37f-7c00-4ecc-b261-17c3e427a8a2"
+imageCount: 14
+lastUpdated: "01/24/25"
+pageDescription: "Aortic Aneurysm: Rupture"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Aortic Aneurysm: Rupture"
+pageTitle: "Aortic Aneurysm: Rupture | STATdx"
+enhancedTitle: "Aortic Aneurysm: Rupture"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Aortic Aneurysm: Rupture"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Dilated aorta with disruption through all 3 layers of aortic wall leading to extravasation of blood into surrounding structures
+ - May occur in setting of acute aortic syndrome where intimal injury is precipitating factor that leads to disruption of wall containing residual medial and adventitia
+- ## Imaging
+
+
+ - Risk of rupture or dissection often linked to aneurysm size, morphology, and rate of growth
+ - Ascending aortic aneurysms (AAs), asymptomatic
+ - In patients with sporadic root and ascending AAs, repair considered with aneurysm size ≥ 5 cm
+ - In patients with genetic aortopathy, repair considered if AA diameter is ≥ 4 cm
+ - Descending thoracic aorta, asymptomatic
+ - In patients with intact descending thoracic aneurysm, repair recommended if diameter ≥ 5.5 cm
+ - In patients with high risk of rupture, repair recommended if diameter ≥ 5 cm
+ - Abdominal aortic aneurysm (AAA), asymptomatic
+ - Elective repair with AAA diameter of ≥ 5.5 cm and ≥ 5 cm in men and women, respectively
+ - Symptomatic patients should undergo repair regardless of size
+ - Depending on rate of growth, repair recommended with
+ - Sporadic thoracic aneurysms and growth rate ≥ 0.5 cm in 1 year or ≥ 0.3 cm per year in 2 consecutive years
+ - Genetic aortopathy or bicuspid aortic valve and growth ≥ 0.3 cm in 1 year
+ - AAA growing ≥ 1 cm in 1 year
+ - CTA findings of rupture
+ - Hemomediastinum, hemopericardium, or hemothorax, hemoperitoneum depending on site
+ - Focal discontinuity in aneurysm wall or disruption of otherwise circumferential aortic calcifications
+- ## Top Differential Diagnoses
+
+
+ - AA without rupture
+ - Acute aortic syndrome with rupture
+ - Aortic pseudoaneurysm
+ - Large vessel vasculitis or inflammatory aortitis (IgG4 or Erdheim-Chester disease)
+- ## Clinical Issues
+
+
+ - Risk factors for thoracic aortic aneurysm rupture include large diameter (especially > 6.0 cm), rapid aneurysm growth (≥ 0.5 cm/year), clinical symptoms, hereditary or infectious etiology, saccular morphology, and female sex
+ - Ascending aortic aneurysm with rupture
+ - Open repair
+ - Arch aneurysm with rupture
+ - Open repair with arch replacement
+ - Descending thoracic or abdominal aortic aneurysm with rupture
+ - Thoracic endovascular aortic repair (TEVAR) or open surgical repair
+
+# TERMINOLOGY
+
+- ## Definitions
+
+
+ - Dilated aorta with disruption through all 3 layers of aortic wall leading to extravasation of blood into surrounding structures
+ - May occur in setting of acute aortic syndrome where intimal injury is precipitating factor that leads to disruption of wall containing residual medial and adventitia
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Enlarged thoracic aorta with associated hemomediastinum, hemopericardium, or hemothorax is highly suggestive of ruptured thoracic aortic aneurysm (TAA)
+ - ### Location
+
+
+ - Abdominal aortic aneurysm (AAA) > TAA
+ - TAA most common in ascending thoracic aorta
+ - However, most ruptures involve AAA descending TAA
+ - ### Size
+
+
+ - Risk of rupture or dissection often linked to aneurysm size, morphology, and rate of growth
+ - Thoracic aneurysms
+ - Aneurysm ≥ 6 cm was significantly associated with risk of rupture or dissection
+ - Yearly rate of rupture or dissection ≥ 6 cm ranges from 10-15%
+ - At ≥ 7 cm, rate dramatically increases to > 40%
+ - Ascending aortic aneurysms (AAs)
+ - For sporadic aortic root and ascending AAs, repair now recommended in certain patients with aneurysm size ≥ 5 cm
+ - Patients with genetic disorders, such as Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome, and familial TAA &/or dissection, repair should be considered if AA diameter is 4.0-5.0 cm
+ - Descending thoracic aorta
+ - In patients with intact descending thoracic aneurysm, repair recommended if diameter ≥ 5.5 cm
+ - In patients with high risk of rupture, repair recommended if diameter ≥ 5 cm
+ - Risk of rupture includes genetic aortopathy, aneurysm causing symptoms, saccular aneurysm, female sex, concern for mycotic aneurysm/pseudoaneurysm, and growth rate ≥ 0.5 cm in 1 year
+ - AAA
+ - Risk of 5-year rupture 20-40% with aneurysms > 5 cm
+ - Elective repair for asymptomatic patients with AAA diameter of ≥ 5.5 cm and ≥ 5 cm in men and women, respectively
+ - For symptomatic patients, should be repaired regardless of size
+ - TAA is commonly defined as maximal thoracic diameter ≥ 4.0 cm
+ - Some define TAA as maximal thoracic diameter ≥ 4.5 cm and maximal thoracic diameter ≥ 4.0 and < 4.5 cm as "dilated"
+ - In reality, upper limits of normal is dependent upon age, sex, body size, and aortic segment
+ - To account for this, z-scores and indexed metrics have been introduced, though adoption in adult populations has been limited
+ - Measurements should be made perpendicular to long axis of aorta, either using double oblique multiplanar reconstruction or centerline CPR to avoid overestimation of diameter due to oblique measurement
+ - Measurements should be reported at sinuses of Valsalva, sinotubular junction, midascending aorta, proximal arch, midarch, proximal and middescending thoracic aorta, and diaphragmatic hiatus, in addition to measurements at most aneurysmal segment
+ - Sinus of Valsalva measurements may be made from sinus-to-sinus or cusp-to-commissure
+ - As sinus-to-sinus measurements tend to be larger than cusp-to-commissure measurements, method used should be specified
+ - If sinuses are largely symmetric, single averaged measurement may be reported
+ - If sinuses are asymmetric, all 3 measurements should be reported
+ - Rate of growth should be assessed when serial imaging is available
+ - ### Morphology
+
+
+ - May be fusiform or saccular with former being more common and latter associated with increased risk of rupture
+ - Wall calcification and mural thrombus are common and may be extensive
+ - Aortic tortuosity may complicate surgical approach and should be described
+ - Rate of growth
+ - Repair recommended for those with sporadic thoracic aneurysms and growth rate ≥ 0.5 cm in 1 year or ≥ 0.3 cm per year in 2 consecutive years
+ - Repair recommended for those with heritable thoracic aortic disease or bicuspid aortic valve and growth ≥ 0.3 cm in 1 year
+ - Repair recommended if AAA is rapidly expanding ≥ 1 cm in 1 year
+- ## Radiographic Findings
+
+
+ - Mediastinal widening may be seen in ruptured or unruptured TAA but is often difficult to appreciate
+ - Pleural effusion (hemothorax) if ruptured into pleural space
+ - Pericardial effusion (hemopericardium) if ruptured into pericardial space
+ - Following thoracic endovascular aortic repair (TEVAR), endograft migration, kinking, or fracture may be identified
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - In patients unable to receive contrast, TAA morphology and diameter may be assessed on NECT
+ - Signs of frank or impending rupture, such as hemomediastinum and intramural hematoma, are both evident on NECT
+ - Following TEVAR, NECT is also useful for distinguishing calcified mural thrombus from endoleak
+ - ### CTA
+
+
+ - Impending, contained, or frank rupture
+ - Hemomediastinum, hemopericardium, or hemothorax depending on location of rupture
+ - Focal discontinuity in aneurysm wall or disruption of otherwise circumferential aortic calcifications
+ - Dense crescent sign: Intramural hematoma or dissection of acute blood into mural thrombus
+ - Lysis of thrombus with expansion of contrast-enhanced flow lumen
+- ## MR Findings
+
+
+ - Can provide much of same information as CTA without ionizing radiation
+ - Following TEVAR, MR is most useful in setting of nickel titanium (nitinol) grafts, which do not produce susceptibility artifacts
+ - LGE of excluded aneurysm sac is sensitive for detecting endoleaks
+ - Time-resolved MR angiography and 4D phase-contrast MR (4D flow) are useful in further characterizing endoleaks
+- ## Echocardiographic Findings
+
+
+ - Transthoracic echocardiography can evaluate aortic root and proximal ascending thoracic aorta as well as complications, such as aortic regurgitation, pericardial effusion, and pericardial tamponade, but is limited by acoustic windows and operator dependence
+ - Transesophageal echocardiography can evaluate entire thoracic aorta with exception of portion of distal ascending aorta and proximal arch, which may be obscured by tracheobronchial tree
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - NECT + CTA
+ - ### Protocol advice
+
+
+ - CTs should be reconstructed using thin (submillimeter) slices to allow for high-quality multiplanar reconstruction (MPRs)
+ - NECT is useful for identifying blood products, and, following endovascular repair, for distinguishing calcified mural thrombus from endoleak
+ - ECG gating is crucial for accurate evaluation aortic root and ascending aorta
+ - If ECG gating is performed retrospectively, multiphase images may be reconstructed enabling assessment of aortic valve function
+ - Delayed imaging is useful for fully opacifying flow lumen in very large aneurysms and for identifying endoleaks occult on arterial phase
+ - Screening
+ - In certain high-risk groups, screening should be performed in patients with family history of TAA or bicuspid aortic valve, personal history of aneurysm elsewhere, or clinical evidence of associated genetic syndrome
+ - Screening should image entire aorta either by CTA or MRA
+ - Screening echocardiography is often indicated
+ - Surveillance
+ - Measurements should be performed at same location, measurement technique, and imaging modality
+ - Patients with newly identified TAA or those near treatment threshold should be imaged more frequently (every 6 months)
+ - Patients with stable TAA may be imaged less frequently (every 1-3 years according to individual risk)
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Aortic Aneurysm Without Rupture
+
+
+ - AAs are not uncommon, especially in older patients
+ - Coexistent pericardial and pleural effusions are not uncommon in patients with TAA due to underlying cardiovascular disease
+ - In some patients, TAAs with complex ulcerated plaque with coexistent simple transudative pleural and pericardial effusions could potentially mimic rupture
+- ## Acute Aortic Syndrome With Rupture
+
+
+ - Aortic dissection, intramural hematoma, or penetrating atherosclerotic ulcer can all lead to aortic rupture
+ - Presence of intimal injury is characteristic of AAS
+ - If rupture occurs in AAS, it is due to disruption of residual media and overlying adventitia
+ - In AAs with rupture, rupture is through all 3 layers of aortic wall
+- ## Aortic Pseudoaneurysm
+
+
+ - Aortic dilation contained by overlying adventitia due to vascular injury
+ - Commonly secondary to trauma, surgery, or infection
+ - More likely to be saccular, able to expand, and change shape rapidly
+- ## Large Vessel Vasculitis or Inflammatory Aortitis (IgG4 or Erdheim-Chester Disease)
+
+
+ - Patients often symptomatic with chest/back pain, weight loss, and elevated inflammatory markers
+ - Areas of stenosis often with coexistent aneurysm
+ - Circumferential wall thickening in areas, often associated with enhancement
+ - Inflammation in periaortic fat could mimic rupture
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - Cystic medial degeneration results in progressive loss of elastin in tunica media
+ - Aortic wall stress increases with aortic diameter (governed by Laplace's Law)
+ - Rupture may occur in TAA with disruption of all 3 layers of aortic wall
+ - Rupture may also occur in setting of acute aortic syndrome
+ - Intimal injury is precipitating cause
+ - Rupture occurs through residual media and adventitia
+ - Recognition of rupture is more important than differentiating between etiologies, although different etiologies may lead to different methods of repair
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Chest pain, hemorrhagic shock, tamponade
+ - Unruptured TAAs are usually asymptomatic and identified incidentally on imaging
+ - ### Other signs/symptoms
+
+
+ - Dysphagia secondary to esophageal compression (dysphagia aortica)
+ - Unilateral hoarseness secondary to mass effect on left recurrent laryngeal nerve (Ortner's syndrome)
+ - Dyspnea secondary to airway compression
+ - Back pain secondary to erosion of vertebral bodies
+- ## Demographics
+
+
+ - TAA occurs in 5-10 per 100,000 person-years
+ - Risk factors for TAA rupture include
+ - Large diameter (especially > 6.0 cm)
+ - Rapid aneurysm growth (≥ 0.5 cm/year)
+ - Saccular morphology
+ - Female sex
+ - Hereditary or infectious etiology
+- ## Natural History & Prognosis
+
+
+ - Once ruptured, usually fatal without repair
+ - Rate of growth and risk of rupture are highly dependent upon underlying etiology as aforementioned
+- ## Treatment
+
+
+ - Ascending AA with rupture
+ - Open repair
+ - Arch aneurysm with rupture
+ - Open repair with arch replacement
+ - Descending thoracic or AAA with rupture
+ - TEVAR or open surgical repair
+ - TEVAR showed lower mortality and reduced risk of paraplegia, stroke, and hypovolemic shock compared to open repair
+
+ 7f6ab856-7757-41e0-8d94-a19884f2f9db
+
+## References
+
+# Selected References
+
+1. [Wyss TR et al: Infective native aortic aneurysm: a Delphi consensus document on treatment, follow up, and definition of cure. Eur J Vasc Endovasc Surg. 67(4):654-61, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38097164%5Bpmid%5D)
+1. [Writing Committee Members et al: 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on clinical practice guidelines. J Thorac Cardiovasc Surg. 166(5):e182-331, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37389507%5Bpmid%5D)
+1. [Isselbacher EM et al: 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on clinical practice guidelines. Circulation. 146(24):e334-482, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36322642%5Bpmid%5D)
+1. [Senser EM et al: Thoracic aortic aneurysm: A Clinical Review. Cardiol Clin. 39(4):505-15, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34686263%5Bpmid%5D)
+1. [Swerdlow NJ et al: Stroke rate after endovascular aortic interventions in the Society for Vascular Surgery Vascular Quality Initiative. J Vasc Surg. 72(5):1593-601, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32249044%5Bpmid%5D)
+1. [Bhave NM et al: Multimodality imaging of thoracic aortic diseases in adults. JACC Cardiovasc Imaging. 11(6):902-19, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29880113%5Bpmid%5D)
+1. [Kim JB et al: Risk of rupture or dissection in descending thoracic aortic aneurysm. Circulation. 132(17):1620-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26338955%5Bpmid%5D)
+1. [Jonker FH et al: Open surgery versus endovascular repair of ruptured thoracic aortic aneurysms. J Vasc Surg. 53(5):1210-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21296537%5Bpmid%5D)
+1. [Elefteriades JA: Natural history of thoracic aortic aneurysms: indications for surgery, and surgical versus nonsurgical risks. Ann Thorac Surg. 74(5):S1877-80; discussion S1892-8, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12440685%5Bpmid%5D)
+1. [Perko MJ et al: Unoperated aortic aneurysm: a survey of 170 patients. Ann Thorac Surg. 59(5):1204-9, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7733722%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*PA CXR in a 63-year-old man with severe chest and abdominal pain in Mexico 3 weeks earlier (who underwent CTA at that time and was told he had a significant problem, but no surgeon would operate) shows increased density in middle mediastinum extending into the abdomen with a partially loculated right pleural effusion .*
+
+
+*PA CXR in a 63-year-old man with severe chest and abdominal pain in Mexico 3 weeks earlier (who underwent CTA at that time and was told he had a significant problem, but no surgeon would operate) shows increased density in middle mediastinum extending into the abdomen with a partially loculated right pleural effusion .*
+
+
+*CTA in the same patient after he traveled to the USA shows rupture of a large thoracoabdominal aortic aneurysm with right hemothorax and blood in the mediastinum .*
+
+
+*Delayed phase from the CTA in the same patient better shows complex loculated fluid collections in right pleural space and mediastinum due to blood products. The aneurysm is again seen.*
+
+
+*Axial oblique CTA (L) after branch fenestrated endovascular thoracoabdominal aortic aneurysm repair for a ruptured aneurysm shows both type III endoleak at left renal artery and superior mesenteric artery origin . The left kidney is infarcted . DWI image (R) 1 day after repair shows a large PCA territory infarct .*
+
+
+*CTA in a 71-year-old man with chest pain shows an aneurysmal ascending aorta with type A dissection. Mediastinal and pericardial hematoma indicate rupture. The only potential rupture site seen on CTA was a slight contour irregularity , which was confirmed surgically.*
+
+
+*CTA in a 76-year-old woman with severe chest pain shows an aneurysmal aorta with a type B dissection with true and false lumens. The false lumen has ruptured , leading to extensive mediastinal hematoma and hemothorax .*
+
+
+*Axial CT in an 83-year-old man with severe chest pain shows a thoracic aortic aneurysm with mild irregularity of the right wall with surrounding mediastinal hematoma and large right hemothorax . Rupture was confirmed surgically.*
+
+
+*PA radiograph in a man in his '80s who presented with streptococcus pneumonia bacteremia and left shoulder pain demonstrates an enlarged aortic contour concerning for thoracic aortic aneurysm. CTA was recommended.*
+
+
+*CTA in the same patient shows a large, multilobulated saccular pseudoaneurysm arising from the lateral aspect of the aortic arch with surrounding soft tissue , concerning for mycotic aneurysm given the known bacteremia.*
+
+
+*Curved planar reconstruction of the aorta in the same patient shows the size and extent of the large, lobulated saccular mycotic pseudoaneurysm . Differentiation between a mycotic pseudoaneurysm and ruptured aneurysm can be difficult without appropriate history.*
+
+
+### Additional Images
+
+
+*CTA in a woman in her 80's with a distant history of coronary artery bypass grafting (CABG) presents with several days of chest pain confirms median sternotomy and post CABG changes . The ascending aorta is aneurysmal with dissection involving both the ascending and descending thoracic aorta.*
+
+
+*Inferior and oblique CTA in the same patient shows there is a large mediastinal hematoma communicating with the aneurysmal ascending thoracic aorta via a focal discontinuity, and fistulation with the right ventricular outflow tract , compatible with rupture.*
+
+
+*NECT in a man in his 80's who presented with chest pain, AMS, and shock shows an aneurysmal ascending aorta with a large, hyperattenuating intramural hematoma .*
+
+
+*Slightly inferior NECT following contrast administration in the same patient shows an ascending thoracic aortic aneurysm and mural thickening representing intramural hematoma as well as large volume hemopericardium , compatible with rupture into the pericardial space.*
+
diff --git a/docs_md/articles/aortic-dissection_57e3428e-1f18-4f38-95c6-f7fe2d93c00a.md b/docs_md/articles/aortic-dissection_57e3428e-1f18-4f38-95c6-f7fe2d93c00a.md
new file mode 100644
index 0000000..4a21065
--- /dev/null
+++ b/docs_md/articles/aortic-dissection_57e3428e-1f18-4f38-95c6-f7fe2d93c00a.md
@@ -0,0 +1,477 @@
+---
+title: "Aortic Dissection"
+docid: "57e3428e-1f18-4f38-95c6-f7fe2d93c00a"
+authors:
+ - key: "770e1d77-2287-436e-910b-48232afc7842"
+ value: "Prabhakar Rajiah, MBBS, MD, FACR, FRCR, FACC, FAHA, FSCCT"
+ - key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
+ value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Aortic Dissection"
+ slug: "aortic-dissection"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "e9f1c786-adf4-4475-8ac7-746741e30004"
+imageCount: 29
+lastUpdated: "12/19/24"
+pageDescription: "Aortic Dissection"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Aortic Dissection"
+pageTitle: "Aortic Dissection | STATdx"
+enhancedTitle: "Aortic Dissection"
+type: "DX"
+references: true
+cases: 1
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Aortic Dissection"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Blood enters media of aortic wall through intimal defect and splits wall longitudinally
+- ## Imaging
+
+
+ - 2 distinct lumina (false and true) with interposed intimal flap
+ - False lumen: Larger cross-sectional area, beak sign, cobweb sign, thrombosis, and delayed enhancement
+ - True lumen: Continuity with undissected portion of aorta and smaller cross-sectional area
+ - Radiograph: Progressive aortic enlargement, widened mediastinum (> 8 cm), and abnormal (blunted) aortic knob
+ - CT: Highly accurate
+ - Slightly less accurate for ascending aorta unless ECG-gated study
+ - MR: Well-suited for follow-up
+ - Transesophageal echocardiography: Operator dependent and with limited field of view
+- ## Top Differential Diagnoses
+
+
+ - Thrombosed aneurysm
+ - Aortic wall hematoma
+ - Syndromes associated with aortic dissection
+- ## Pathology
+
+
+ - Media degeneration associated with many diseases and syphilitic aortitis, crack cocaine use, and iatrogenic (catheter angiography, cardiac surgery, valve replacements)
+ - Tear in intimal layer leading to formation and propagation of subintimal hematoma
+- ## Clinical Issues
+
+
+ - Type A: Surgery due to involvement of aortic root
+ - Type B: Medical control of hypertension is standard
+ - Percutaneous therapy for complicated nonsurgical patients with type B dissections
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Aortic dissection (AD)
+- ## Definitions
+
+
+ - Blood enters media of aortic wall through intimal defect and splits wall longitudinally
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - 2 distinct lumina with interposed intimal flap: Double-barrel aorta
+ - Displacement of intimal calcification or compression/distortion of aortic lumen
+ - ### Location
+
+
+ - Stanford classification
+ - Type A (60%): Involves ascending aorta
+ - 90% within 10 cm of aortic valve
+ - Type B (40%): Does not involve ascending aorta
+ - DeBakey classification
+ - Type I: Originates in ascending aorta, involves at least aortic arch, and may involve descending aorta
+ - Type II: Originates in and confined to ascending aorta
+ - Type III: Originates in descending aorta; IIIa: Limited to descending aorta; IIIb: Extends below diaphragm
+ - Additional imaging should evaluate involvement of great vessels and celiac, renal, superior mesenteric, and iliac arteries
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - Often abnormal CXR but nonspecific
+ - 25% are normal
+ - Widened mediastinum (> 8 cm)
+ - Abnormal (blunted) aortic knob in 66%
+ - Ring sign: > 5-mm displacement of aortic lumen past calcified aortic intima
+ - Apparent displacement of intimal calcification may be projectional artifact
+ - Left apical cap, tracheal deviation, depression of left mainstem bronchus, esophageal deviation, loss of paratracheal stripe, pericardial effusion, and hemothorax
+ - Progressive aortic enlargement on serial images
+ - Enlarged arch is not specific for diagnosis; usually results from hypertension or atherosclerosis
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - Widening of aorta, irregularity of aortic wall, and intramural or periaortic acute thrombus
+ - Hyperattenuating mediastinal, pericardial, or pleural fluid (blood)
+ - Internally displaced intimal calcification
+ - ### CECT
+
+
+ - ECG gating allows delineation of proximal extent of intimal flap in relation to aortic valve and coronary ostia
+ - Intimal flap separates true and false lumina
+ - True vs. false lumen
+ - False lumen is larger than true lumen
+ - False lumen wedges around true lumen, beak sign
+ - Cobweb sign of false lumen due to collagenous media remnants
+ - False lumen contrast attenuation
+ - Usually, slower flow with lower contrast attenuation on arterial phase
+ - Thrombosis leads to nonenhancement
+ - Complete thrombosis/reduced flow in false lumen decreases risk of aortic dilatation
+ - Windsock appearance: Dissection of entire intima leads to circumferential intimal flap
+ - Filiform-shaped, narrow true lumen
+ - Leads to intimointimal intussusception
+ - Triple-channel dissection: Secondary dissection within 1 channel (Mercedes-Benz sign)
+ - Complications of AD
+ - Rupture into pericardium, left pleural cavity, mediastinum, right ventricle, left atrium, vena cavae, pulmonary arteries
+ - Pericardial tamponade
+ - Acute aortic regurgitation
+ - Major aortic branch obstruction
+ - Visceral or extremity malperfusion
+ - Compression of true lumen by false lumen
+ - Intimal flap in renal, celiac, mesenteric, or extremity arteries
+ - Renal: Absence of nephrographic effect in late phase of CT
+ - Retrograde dissection into aortic arch
+ - In 27% of type B dissections; higher mortality (43%)
+- ## MR Findings
+
+
+ - ### T1WI
+
+
+ - Site of intimal tear; type and extent of dissection
+ - Signal intensity within false lumen is variable
+ - Depends on blood flow; thrombus: Presence, age, composition; and pulse sequence
+ - Usually signal is seen due to slow flow
+ - Flow void in true lumen
+ - Useful in abdominal arterial involvement and monitoring of progression of dissection and aneurysm formation
+ - Aortoannular ectasia
+ - ### MRA
+
+
+ - Demonstrates flap, true and false lumen
+ - Extent of flap into branch vessels can be seen
+ - End-organ perfusion can be evaluated
+ - ### SSFP cine
+
+
+ - Intimal flap: Intermediate signal
+ - Reentry site: Low-signal turbulent flow between lumina
+ - False lumen with blood flow/thrombus: Medium to low signal
+ - Aortic insufficiency and pericardial rupture
+ - Phase-contrast imaging
+ - Flow dynamics of lumina can be evaluated
+ - 4D flow MR
+ - Detailed evaluation of hemodynamics
+ - Flow/thrombosis of false lumen evaluated more reliably than CT
+ - Retrograde flow is less in true than false lumen
+ - Helical flow in false lumen is sign of aortic expansion
+ - Progressive aortic enlargement evaluated by MR/cardiac CT
+ - Features predictive of descending aortic enlargement
+ - > 10-mm primary intimal tear in descending aorta
+ - Descending aorta > 35 mm
+ - False lumen > 22 mm in proximal descending aorta
+ - False lumen > 2/3 of total descending aorta
+ - Partially thrombosed distal false lumen
+ - Distal suture line leak
+ - Helical flow in false lumen
+ - Remodeling: 10% volumetric difference of aorta
+ - Favorable remodeling: > 10% decrease in false lumen or > 10% increase in true lumen diameter
+ - Unfavorable remodeling: Increased diameter of false and decreased diameter of true lumen
+ - Due to persistent refilling of false lumen
+ - After surgical repair of type A: Persistent arch intimal tear; leak of distal graft anastomosis; refilling from false lumen of dissected aortic arch vessel
+ - After thoracic endovascular aortic repair (TEVAR): Incomplete seal of proximal landing zone due to aortic tortuosity; arch branch stump, supraarch chimney stent or TEVAR technique
+- ## Echocardiographic Findings
+
+
+ - ### Echocardiogram
+
+
+ - Transesophageal echocardiography (TEE)
+ - Undulating intimal flap within aortic lumen
+ - May identify entry site, presence of false lumen thrombus, abnormal flow characteristics, involvement of coronary and arch vessels, pericardial effusion, and aortic valve regurgitation
+ - False-positives: May occur if calcified aorta mimics intimal flap
+ - ### Color Doppler
+
+
+ - Identifies flow in false lumen, site of intimal tear, and presence or absence of aortic insufficiency
+- ## Angiographic Findings
+
+
+ - Conventional
+ - False lumen is visualized in 87%, intimal flap in 70%, and site of intimal tear in 56%
+ - May show site of intimal tears, aortic valve regurgitation, coronary artery involvement, and filling of branch vessels
+ - Indirect signs of AD: Compression of true lumen by false lumen and abnormal appearance of branch vessel origins
+ - False-negative angiogram may occur due to thrombosis of false lumen
+- ## Imaging Recommendations
+
+
+ - CT: Highly accurate and rapid; imaging procedure of choice
+ - ECG gating or high-pitch helical mode is necessary to avoid risk of false-positives in aortic root
+ - MR: Better suited in nonemergent setting: Anatomic information; flow dynamics of false and true lumen
+ - TEE: Possible in most patients, including unstable
+ - Highly dependent on operator experience
+ - Not used if esophageal varicosities or stenosis
+ - Limited view of dissection
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Thrombosed Aneurysm
+
+
+ - Large aorta and aortic lumen size
+- [Intramural Hematoma](/document/aortic-intramural-hematoma/128bc4cc-a26d-47d5-90e7-b1a1f608e657)
+ - Hemorrhage within wall with no identifiable intimal tear, flap, or false lumen
+ - Caused by bleeding from vasa vasorum into media
+- [Penetrating Aortic Ulcer](/document/penetrating-atherosclerotic-ulcer/63f6cba2-2200-456a-8d03-ac3111e420c8)
+ - Perforation of aortic wall in region of ulcerated atherosclerotic plaque
+ - Most common in descending aorta
+ - May progress to dissection
+- ## Syndromes and Conditions Associated With Aortic Dissection
+
+
+ - Marfan syndrome, Ehlers-Danlos syndrome, bicuspid aortic valve
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Medial degeneration is associated with many diseases that predispose to dissection
+ - Hypertension (70%), atherosclerosis
+ - Structural collagen disorder (Marfan or Ehlers-Danlos syndrome)
+ - Congenital disease (aortic coarctation; bicuspid or unicuspid valve); pregnancy
+ - Syphilitic aortitis, crack cocaine use, and iatrogenic (catheter angiography, cardiac surgery, valve replacements)
+ - Dissections almost exclusively originate in thoracic aorta and secondarily involve abdominal aorta
+ - SMMHC marker is helpful in diagnosis
+- ## Gross Pathologic & Surgical Features
+
+
+ - Intimal tear: Formation/propagation of subintimal hematoma
+ - 5-10% are without intimal tear; dissection is attributed to rupture of aortic vasa vasorum
+ - Diseases that weaken aortic wall predispose to AD
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Sudden onset of ripping or tearing chest pain
+ - Anterior chest pain: Ascending AD
+ - Neck or jaw pain: Aortic arch dissection
+ - Back tearing or ripping pain: Descending AD
+ - Myocardial infarction
+ - 50% of AD: Women < 40 years, related to pregnancy
+ - Sudden onset of aortic insufficiency, neurologic deficits in 20% of cases, and ischemic extremity
+- ## Demographics
+
+
+ - ### Age
+
+
+ - 75% in 40-70 years; peak at 50-65 years
+ - ### Sex
+
+
+ - M:F = 3:1
+ - ### Ethnicity
+
+
+ - Black > White > Asian patients
+- ## Natural History & Prognosis
+
+
+ - Acute AD: < 2 weeks from initial onset of symptoms; subacute: 2 weeks to 3 months; chronic: > 3 months
+ - Complications: Rupture, cardiac tamponade, aortic insufficiency with acute heart failure, occlusion of coronary or supraaortic vessels
+ - 21% of patients die before hospital admission
+ - If untreated, 33% die within 24 hours; 50% within 48 hours
+ - < 10% of untreated patients with type A live 1 year
+- ## Treatment
+
+
+ - Type A: Surgery due to involvement of aortic root
+ - Ascending aorta only: Supracoronary ascending aorta replacement, intimal resection, aortic valve repair/replacement
+ - Arch/hemiarch: Antegrade thoracic endovascular aortic repair (TEVAR), elephant trunk, frozen elephant trunk, or multibranched arch graft
+ - Bentall procedure: If valve cusps are dissected or with Marfan syndrome with preexisting root aneurysm
+ - Type B: Conservative treatment is standard (Rx of hypertension)
+ - Surgery in complicated cases
+ - Mesenteric, renal, extremity ischemia
+ - Rupture, aneurysmal enlargement of false lumen; descending aorta > 6 cm
+ - Hemodynamic instability, pseudocoarctation syndrome, distal embolization
+ - Percutaneous therapy (aortic stent graft or fenestration of flap) for complicated nonsurgical patients with type B dissections
+ - Visceral malperfusion, dilated aortic arch or proximal descending aorta (≥ 4.5 cm), impending rupture
+ - Type A mortality: 60% of medically treated; 30% of surgically treated
+ - Type B mortality: 10% of medically treated; 30% of surgically treated
+ - DISSECT classification system for endovascular decision making
+ - Based on 6 features: **D**uration, **i**ntimal tear, **s**ize of dissected aorta, **s**egmental **e**xtent of involvement, **c**linical complications, **t**hrombosis of false lumen
+ - TEVAR in subacute/chronic setting
+ - Descending aorta > 5.5 cm, > 5-mm growth/6 months, refractory pain, impending aortic rupture
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - AD in patient with acute chest pain
+ - ECG-gated CTA in acute AD; MRA for follow-up
+- ## Image Interpretation Pearls
+
+
+ - Identify origin of intimal flap, extent of dissection, and origin of aortic branches from true or false lumen
+
+ b7ed7b4f-f032-49ba-9686-ee52cc5a7b35
+
+## References
+
+# Selected References
+
+1. [Writing Committee Members et al: 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: a report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 80(24):e223-393, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36334952%5Bpmid%5D)
+1. [Ko JP et al: Chest CT angiography for acute aortic pathologic conditions: pearls and pitfalls. Radiographics. 41(2):399-424, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33646903%5Bpmid%5D)
+1. [Murillo H et al: Aortic dissection and other acute aortic syndromes: diagnostic imaging findings from acute to chronic longitudinal progression. Radiographics. 41(2):425-46, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33646901%5Bpmid%5D)
+1. [Baliyan V et al: Acute aortic syndromes and aortic emergencies. Cardiovasc Diagn Ther. 8(Suppl 1):S82-96, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29850421%5Bpmid%5D)
+1. [Di Eusanio M et al: Clinical presentation, management, and short-term outcome of patients with type A acute dissection complicated by mesenteric malperfusion: observations from the International Registry of Acute Aortic Dissection. J Thorac Cardiovasc Surg. 145(2):385-90, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=22341418%5Bpmid%5D)
+1. [Clough RE et al: A new imaging method for assessment of aortic dissection using four-dimensional phase contrast magnetic resonance imaging. J Vasc Surg. 55(4):914-23, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22386146%5Bpmid%5D)
+1. [Amano Y et al: Time-resolved three-dimensional magnetic resonance velocity mapping of chronic thoracic aortic dissection: a preliminary investigation. Magn Reson Med Sci. 10(2):93-9, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21720111%5Bpmid%5D)
+1. [Rogers AM et al: Sensitivity of the aortic dissection detection risk score, a novel guideline-based tool for identification of acute aortic dissection at initial presentation: results from the international registry of acute aortic dissection. Circulation. 123(20):2213-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21555704%5Bpmid%5D)
+1. [McMahon MA et al: Multidetector CT of aortic dissection: a pictorial review. Radiographics. 30(2):445-60, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20228328%5Bpmid%5D)
+1. [Liu Q et al: Three-dimensional contrast-enhanced MR angiography of aortic dissection: a pictorial essay. Radiographics. 27(5):1311-21, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17848693%5Bpmid%5D)
+1. [García A et al: MR angiographic evaluation of complications in surgically treated type A aortic dissection. Radiographics. 26(4):981-92, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16844927%5Bpmid%5D)
+1. [Bortone AS et al: Endovascular treatment of thoracic aortic disease: four years of experience. Circulation. 110(11 Suppl 1):II262-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15364873%5Bpmid%5D)
+1. [Batra P et al: Pitfalls in the diagnosis of thoracic aortic dissection at CT angiography. Radiographics. 20(2):309-20, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10715333%5Bpmid%5D)
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1', 'value': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI'}], 'caseVersionId': '9f6396b1-70ae-4cf3-9985-f5018fc0256a', 'description': 'A type A dissection flap within the ascending aorta (arrow, #1) and high density hemopericardium (open arrows, #1) are evident (#1). Note the dense contrast in the azygos system. There is dissection in the abdominal aorta and superior mesenteric artery (#2). Note that there is dense contrast refluxed and layering in the SVC, due to pericardial tamponade. There is a patent right common iliac artery, and contrast is void in the left common iliac artery (curved arrows, #3), which is occluded by the dissection flap (#3).', 'history': 'Acute onset of tearing pain between scapulas.', 'imagePoolId': 'b20e0be4-906f-477d-9e41-4fd4d7a78023', 'name': 'Type A dissection hemopericardium/tamponade', 'teachingPoint': None, 'demographics': '52 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'c4211efd-db8c-425c-ab52-babc85237512', 'description': 'Typical radiographic and CT features of ascending aortic dissection in Marfan syndrome.\n\nPortable radiograph (#1) shows prominent ascending aorta (arrow). CECT (#2) shows dilatation of ascending aorta (arrows) and intimal flap (open arrow). Coronal reconstruction (#3) shows intimal flap (open arrow) in ascending aortic dissection.', 'history': 'Chest pain.', 'imagePoolId': '94201114-7b96-4ad9-a6e1-9707e4e48bb7', 'name': 'Ascending aortic dissection', 'teachingPoint': None, 'demographics': '34 Years old male'}, {'authors': [{'key': 'd69a0534-8989-4b83-b19d-c3b640f22e53', 'value': 'Megan R. Saettele, MD'}, {'key': '90e33361-cf04-431b-a6fb-4a18e6aa42a1', 'value': 'Joseph P. Koury, MD'}, {'key': '2e78a4ff-418e-46f9-93e4-c6d95d960e35', 'value': 'Brandt C. Wible, MD'}], 'caseVersionId': 'd806de07-6560-4a7e-99ea-6e0c12d137aa', 'description': 'Axial computed tomography images (#1-7) show acute aortic dissection with the intimal flap (arrows) that extends from the aortic root, into the celiac axis (#4) and superior mesenteric artery (#5), and down through the common iliac arteries (#7). This a Stanford type A, DeBakey type I aortic dissection. Sagittal (#8) and coronal (#9) reconstructions again identify the aortic dissection (arrows) extending throughout the length of the aorta and into the common iliac arteries.\n\nComment: Aortic dissections are described according to the Stanford or DeBakey classification systems. Stanford classification divides aortic dissections into either Type A or Type B, with Type A dissections involving the ascending aorta. Type A dissections are more common (60-70%) than Type B dissections, often requiring surgical treatment, and may or may not involve the descending aorta. Type B dissections involve only the descending aorta distal to the origin of the left subclavian artery. Type B dissections can often be managed with medical therapy. The DeBakey classification system divides aortic dissection into type I (involves the ascending and descending aorta), type II (confined to the ascending aorta), and type III (confined to the descending aorta). A key finding on contrast-enhanced CT images, such as was seen in this patient, is an intimal flap between the true and false lumens. It is important to identify the extent of the dissection as well because they can extend cranially to involve the carotid or vertebral vessels or caudally to involve the celiac, SMA, IMA, renal, or iliac vessels.', 'history': 'Patient presented to the emergency room complaining of tearing chest and back pain.', 'imagePoolId': 'e36adebd-8d00-4674-9e0a-f0e1102822b7', 'name': 'Acute aortic dissection type A and B', 'teachingPoint': None, 'demographics': '59 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': '857e6cd9-44b3-4f9f-8d73-dcc0d6001d8a', 'description': 'Typical CT features of chronic consolidation from lipoid pneumonia.\n\nCECT (#1,2) shows multifocal consolidation particularly in the right middle lobe (arrows). CECT (#3-9) shows descending aortic aneurysm (arrows). Consolidated lung is of fat density (open arrows, #5-8). Mineral oil aspiration.', 'history': 'Cough.', 'imagePoolId': 'c37cbfc6-9157-46d1-bd7f-9c4920969fb8', 'name': 'Chronic consolidation', 'teachingPoint': None, 'demographics': '65 Years old male'}, {'authors': [{'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}, {'key': '3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1', 'value': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI'}], 'caseVersionId': '229680f9-ecb1-4f64-a6ba-0f0715b5b91f', 'description': 'Axial (#1) and coronal (#2) images from a contrast-enhanced CTA show a dissection flap (arrows) in the ascending aorta, consistent with a type A aortic dissection. Axial VR image (#3) shows that the dissection flap (arrow) does not extend to the distal aspect of the aortic arch. \n\nComment: Typically, type A aortic dissections are treated surgically given their mortal complications, such as cardiac ischemia (from extension into coronary arteries), pericardial tamponade (from rupture into pericardium), and acute heart failure (from involvement of the aortic valve leading to uncompensated aortic insufficiency).', 'history': 'Patient with history of intermittent chest pain.', 'imagePoolId': 'f4acd209-2f91-458a-910c-a4617997c7bf', 'name': 'Type A aortic dissection', 'teachingPoint': None, 'demographics': '66 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Frontal chest radiograph shows subtle abnormal contour of the ascending aorta, suggestive of ascending aortic aneurysm , but this finding is nonspecific. The descending aorta is tortuous, which can be seen in systemic hypertension.*
+
+
+*Frontal chest radiograph shows subtle abnormal contour of the ascending aorta, suggestive of ascending aortic aneurysm , but this finding is nonspecific. The descending aorta is tortuous, which can be seen in systemic hypertension.*
+
+
+*Frontal radiograph in the same patient presenting a few months later with acute chest pain shows increased abnormal contour and abnormal widening of the mediastinum , suggestive of aortic dissection.*
+
+
+*Axial CTA through the thorax in a patient with Stanford type A dissection shows a nearly circumferential dissection flap involving the ascending and descending aortas. Note the small true lumen in the ascending aorta . The intimal flap can be quite mobile on gated cine imaging.*
+
+
+*Axial CTA at the level of arch in the same patient shows extension of the dissection flap into the right brachiocephalic artery . Note the displaced intimal calcification in the descending aorta .*
+
+
+*DeBakey type I and Stanford class A include dissections that involve the ascending aorta. DeBakey type II is confined to the ascending aorta, and type I extends beyond. DeBakey type III dissections are confined to the descending aorta. Stanford class B includes all dissections not involving the ascending aorta (involving arch &/or descending aorta).*
+
+
+*"Candy cane" sagittal oblique (left) and axial (right) CTA views show Stanford type A aortic dissection with partially thrombosed false lumen .*
+
+
+*Axial and VR CTA show Stanford type B aortic dissection involving descending thoracic and abdominal aorta. The true lumen is smaller and located laterally. Note the infrarenal abdominal aortic aneurysm .*
+
+
+*Transthoracic echocardiogram shows ascending aortic dissection with dissection flap (white interface ) separating true and false lumina. Most of the false lumen is hyperechoic, suggesting thrombosis . The small, anechoic portion corresponds to a patent false lumen .*
+
+
+*Axial CECT shows acute type A aortic dissection with flap that is convex toward false lumen . Note that a beak sign is present in the false lumen. The appearance of a beak sign is attributed to the higher systolic pressure in the true lumen.*
+
+
+*Axial MRA shows chronic type B aortic dissection with partial thrombosis of the false lumen (hypointense area) . Compared with the false lumen, the true lumen is relatively small . Note the perfused portion of the false lumen .*
+
+
+*Axial NECT, CTA, and MR in a patient with type B aortic dissection are shown. The aortic dissection is not seen on NECT. Axial unenhanced MR shows aortic dissection on HASTE (dark blood) and SSFP (bright blood) , an advantage over NECT.*
+
+
+*Axial NECT and CTA show chronic descending thoracic aortic dissection with a flat dissection flap. Outer wall calcification and thrombus are present in the false lumen .*
+
+
+*Axial CECT shows a high-attenuation pericardial effusion , consistent with a hemopericardium, as well as bilateral trace pleural effusions . The dissection flap is clearly visible in the descending thoracic aorta .*
+
+
+*Axial CTA shows abdominal aortic dissection with Mercedes-Benz sign relating to a triple-channel dissection resulting in 2 false lumina . One of the 2 intimal flaps extends to the origin of the right renal artery .*
+
+
+*Axial (left) and oblique (right) CTA demonstrate a type A aortic dissection involving the right coronary artery ostium in a patient status post percutaneous aortic valve implantation/replacement (TAVI/R).*
+
+
+*Axial CTA shows an abdominal aortic dissection . Note that the dissection flap does extend into the superior mesenteric artery , which remains well perfused.*
+
+
+*Axial CTA shows partial eccentric thrombosis of the false lumen in a chronic aortic dissection. The celiac artery arises from the false lumen and is thrombosed.*
+
+
+*Oblique MIP CTA shows dissection of the descending thoracic and abdominal aorta. Note main and accessory renal arteries arising from the partially thrombosed false lumen and a subtotal infarct of the left kidney . Main and accessory renal arteries demonstrate partial flow.*
+
+
+*Axial CTA shows a dissection flap extending into the left common carotid artery . The true lumen is smaller and enhances more than the false lumen .*
+
+
+*Axial (left) and coronal (right) CTA show atypical appearance of an aortic dissection involving the entire intima of the thoracic aorta circumferentially. This phenomenon may subsequently lead to intimointimal intussusception.*
+
+
+*Sagittal MRA shows an aortic dissection, which is seen starting from the arch distal to the origin of the left subclavian artery , and extends into the abdominal aorta, extending into the iliac arteries. The true lumen enhances more than the false lumen .*
+
+
+*Coronal MR cine shows the false lumen supplying the left renal artery. The small, compressed true lumen supplies the right renal artery. The false lumen is thrombosed in the infrarenal area.*
+
+
+### Additional Images
+
+
+*Coronal CTA shows marked compression of the true lumen by the false lumen . There is partial thrombosis of the false lumen, although still with some enhancement.*
+
+
+*Coronal CECT shows a dissection flap extending from the aortic arch into the descending aorta. The flap is spiraling down the aorta with involvement of the aortic branches.*
+
+
+*Coronal CTA shows compression of the true lumen in the abdominal aorta . Right renal artery is supplied by the true lumen . Left renal artery originates from the false lumen.*
+
+
+*Oblique catheter angiography shows both the true lumen and the false lumen . There is a flap in the innominate artery and decreased flow in other great vessels .*
+
+
+*Axial CECT shows a chronic dissection of the abdominal aorta. There are 2 regions of perfusion , and the rest of the aorta is thrombosed .*
+
+
+*DSA shows a bizarre appearance of the abdominal aorta. Note marked smooth narrowing of the infrarenal aorta with change in caliber and contrast enhancement. Also note narrowing of the left renal artery , which may be related to involvement by dissection.*
+
+
+*Sagittal CTA shows a dissection. The false lumen compresses the true lumen . The true lumen supplies the celiac axis and superior mesenteric artery .*
+
diff --git a/docs_md/articles/aortic-intramural-abnormality_75d7b37f-bc37-493b-8961-8b2a9001fb94.md b/docs_md/articles/aortic-intramural-abnormality_75d7b37f-bc37-493b-8961-8b2a9001fb94.md
new file mode 100644
index 0000000..decdea6
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+++ b/docs_md/articles/aortic-intramural-abnormality_75d7b37f-bc37-493b-8961-8b2a9001fb94.md
@@ -0,0 +1,136 @@
+---
+title: "Aortic Intramural Abnormality"
+docid: "75d7b37f-bc37-493b-8961-8b2a9001fb94"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Aortic Intramural Abnormality"
+ slug: "aortic-intramural-abnormality"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "8ad4b88e-7101-4be0-a839-eb819f6dfe08"
+imageCount: 9
+lastUpdated: "03/17/22"
+pageDescription: "Aortic Intramural Abnormality"
+pageKeywords: "Cardiac, Differential Diagnosis, Aortic Intramural Abnormality"
+pageTitle: "Aortic Intramural Abnormality | STATdx"
+enhancedTitle: "Aortic Intramural Abnormality"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Aortic Intramural Abnormality"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Aortic wall should measure < 4 mm
+ - Aortic wall should be isointense to lumen
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Atherosclerosis/Adherent Thrombus**
+ - Concentric diffuse involvement vs. spiral involvement of intramural hematoma
+ - Aorta often tortuous with atherosclerotic disease in branch vessels
+ - **Aortic Dissection**
+ - Intimal flap readily seen on CECT as unenhanced line through lumen
+ - Intraluminal calcifications on NECT suggest diagnosis and represent displaced intimal calcifications
+ - "Beak" sign: False lumen side of dissection flap meets outer wall with acute angle
+ - "Cobweb" sign: False lumen traversed by media fibers
+ - Confusion with pulsation artifact at aortic root avoided by inspecting coronal images
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Aortic Intramural Hematoma**
+ - Hyperdense aortic wall compared to lumen when acute, isodense when old
+ - Check LV chamber for hypodense blood to avoid pitfall of confusion anemia
+ - Patient more likely to progress to dissection with coexistence of ulcer-like projections
+ - Most commonly in descending aorta
+ - **Penetrating Atherosclerotic Ulcer**
+ - Luminal irregularity
+ - Must extend beyond expected contour of intima
+ - Outer aortic wall thickening indicates acuity
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Takayasu/Giant Cell Arteritis**
+ - Radiographically indistinguishable, differentiated based on age (Takayasu < 50 years, giant cell > 50 years)
+ - FDG PET can determine active disease
+ - Aortic caliber will be reduced
+ - Subclavian stenosis is hallmark finding
+ - Pulmonary artery strictures and mesenteric vessel stenosis are common
+ - **Radiation**
+ - Vascular calcifications confined to radiation field
+ - Radiation history will be present
+
+## References
+
+# Selected References
+
+1. [Ko JP et al: Chest CT Angiography for acute aortic pathologic conditions: pearls and pitfalls. Radiographics. 41(2):399-424, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33646903%5Bpmid%5D)
+1. [Murillo H et al: Aortic dissection and other acute aortic syndromes: diagnostic imaging findings from acute to chronic longitudinal progression. Radiographics. 41(2):425-46, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33646901%5Bpmid%5D)
+1. [Carroll BJ et al: Imaging for acute aortic syndromes. Heart. 106(3):182-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31822571%5Bpmid%5D)
+1. [Abbara S et al: Thoracic aortic disease: spectrum of multidetector computed tomography imaging findings. J Cardiovasc Comput Tomogr. 1(1):40-54, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=19083876%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Atherosclerosis/Adherent Thrombus**
+*Axial CECT shows a mural thrombus in an otherwise dilated aorta. Note that intimal calcifications are on the outer edge of the thrombus .*
+
+
+**Atherosclerosis/Adherent Thrombus**
+*Axial CECT shows a mural thrombus in an otherwise dilated aorta. Note that intimal calcifications are on the outer edge of the thrombus .*
+
+
+**Aortic Dissection**
+*Axial CECT shows a severely displaced dissection flap, compressing the true lumen and occluding the SMA . Note true lumen and false lumen . This was treated with fenestration.*
+
+
+**Aortic Dissection**
+*Axial CECT shows a dissection flap in the descending aorta with a displaced intimal calcification . The dissection did not involve the arch and was managed medically.*
+
+
+**Aortic Intramural Hematoma**
+*Coronal NECT shows asymmetric thickening and hyperdense aortic wall, representing the hematoma. Note the displaced intimal calcification .*
+
+
+**Aortic Intramural Hematoma**
+*Axial CECT shows hyperdense, thickened aortic wall in a hypertensive patient who presented to the ER with back pain. This finding can be missed on an enhanced exam.*
+
+
+**Penetrating Atherosclerotic Ulcer**
+*Axial CECT shows a small focus of contrast extending beyond the expected aortic wall . Adjacent wall thickening suggested it is likely acute and explains the patient's back pain.*
+
+
+**Takayasu/Giant Cell Arteritis**
+*Axial CECT shows thickened aortic wall with inner intimal calcifications. Although causing aortic narrowing, this may progress to aneurysmal dilation.*
+
+
+**Radiation**
+*Double oblique CECT shows dense aortic and pulmonary artery calcifications in a patient who received prior mediastinal radiation. Vascular calcifications were not present elsewhere. Note that calcifications are spatially confined to the radiation field.*
+
+
+### Additional Images
+
+
+**Aortic Dissection**
+*Axial NECT shows displaced intimal calcifications representing aortic dissection .*
+
diff --git a/docs_md/articles/aortic-intramural-hematoma_128bc4cc-a26d-47d5-90e7-b1a1f608e657.md b/docs_md/articles/aortic-intramural-hematoma_128bc4cc-a26d-47d5-90e7-b1a1f608e657.md
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@@ -0,0 +1,445 @@
+---
+title: "Aortic Intramural Hematoma"
+docid: "128bc4cc-a26d-47d5-90e7-b1a1f608e657"
+authors:
+ - key: "b66f94a2-4335-4ce8-a3ba-8c5527f8774c"
+ value: "Domenico Mastrodicasa, MD"
+ - key: "5de0df07-7b3e-4678-8767-1519e1153f29"
+ value: "Dominik Fleischmann, MD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Aortic Intramural Hematoma"
+ slug: "aortic-intramural-hematoma"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "df9b047f-b83f-46c7-85da-2cecf3b24727"
+imageCount: 23
+lastUpdated: "12/19/24"
+pageDescription: "Aortic Intramural Hematoma"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Aortic Intramural Hematoma"
+pageTitle: "Aortic Intramural Hematoma | STATdx"
+enhancedTitle: "Aortic Intramural Hematoma"
+type: "DX"
+references: true
+ddx: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Aortic Intramural Hematoma"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Intramural hematoma (IMH) (literally blood in wall) in its broadest sense refers to fresh thrombus/clot within aortic wall; term IMH is used clinically (and somewhat confusingly) for 2 different meanings
+ - IMH as aortic dissection (AD) variant such as AD with thrombosed false lumen or AD with small entry and absent reentry tear
+ - IMH as purely descriptive term referring to often localized hematoma ("bruise") in aortic wall, which can be associated with broad range of acute aortic pathologies, including penetrating aortic ulcer, limited intimal tear, traumatic aortic injuries, rupturing aneurysms, iatrogenic dissections, etc.
+- ## Imaging
+
+
+ - NECT: Crescentic or eccentric aortic wall hyperdensity
+ - CECT
+ - Crescentic or eccentric aortic wall thickening
+ - ↓ luminal aortic diameter along (i.e., candy cane) IMH extension
+ - Penetrating aortic ulcer (PAU): Outpouching of contrast beyond expected aortic margin in setting of atherosclerosis; penetrates internal elastic lamina
+ - Ulcer-like projections (ULP): Outpouching of contrast with wide intimal opening (i.e., > 3 mm), limited to intima
+ - PAU appears similar to ULP; PAU is often surrounded by IMH, which has smooth interface with lumen
+ - Intramural blood pool (IBP)
+ - Pool of contrast with small luminal communication (1-2 mm)
+ - Often has communication with aortic branch (e.g., intercostal/lumbar arteries)
+- ## Top Differential Diagnoses
+
+
+ - Aortitis (Takayasu and giant cell arteritis)
+ - Aortic aneurysm with mural thrombus
+- ## Pathology
+
+
+ - Stanford classification
+ - Type A (~ 40%): Ascending ± descending aorta
+ - Type B (~ 60%): Descending aorta
+- ## Clinical Issues
+
+
+ - Abrupt onset of severe chest or back pain, hypertension
+ - Predictors of poorer prognosis and ↑ mortality
+ - Stanford type A
+ - Maximum aortic diameter (ascending: 48-55 mm; descending: 40-41 mm)
+ - IMH thickness 10-11 mm
+ - PAU
+ - Treatment
+ - Type A IMH: Surgical treatment
+ - Type B IMH: Medical treatment and close follow-up
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Intramural hematoma (IMH), aortic dissection (AD)
+- ## Definitions
+
+
+ - IMH (literally: blood in wall) in its broadest sense refers to fresh thrombus/clot within aortic wall; term IMH is used clinically (and somewhat confusingly) for 2 different meanings
+ - IMH as AD variant
+ - AD thrombosed false lumen or AD with small entry and absent reentry tear
+ - IMH as purely descriptive term referring to often localized hematoma ("bruise") in aortic wall
+ - Associated with broad range of acute aortic pathologies, including PAU, LIT, traumatic aortic injuries, rupturing aneurysms, iatrogenic dissections, etc.
+ - IMH (or class 2 AD)
+ - Absent or very small entry tear and absent reentry tear
+ - PAU (or class 4 AD)
+ - Ulcerated atherosclerotic lesion that penetrates internal elastic lamina into media ± IMH
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Aortic wall hyperdensity on NECT can be focal, crescentic, elongated, &/or circumferential
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - Often normal chest radiograph
+ - Interval radiography: Displacement of intimal calcification
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - Focal, crescentic, elongated, &/or circumferential aortic wall hyperattenuation
+ - Nonclotted (flowing) blood → 30-45 HU; acute thrombus → 50-90 HU
+ - In first 7-10 days, acute thrombus is hyperdense relative to blood because it retracts (↑ hemoglobin concentration)
+ - Pitfalls: Anemia, residual contrast medium; left ventricular myocardium can be used as reference
+ - Narrow window width and level settings helpful to recognize hyperattenuating IMH
+ - Can be seen with LIT in ascending aorta
+ - ### CTA
+
+
+ - Crescentic or eccentric aortic wall thickening, smooth lumen-wall interface
+ - ↓ luminal aortic diameter along IMH extension
+ - better seen on long-axis reformations (e.g., candy cane views)
+ - Often, no discrete intimomedial flap seen on CT but can be identified surgically or pathologically in nearly all cases
+ - Identification of intimomedial flap by CT represents coexistent AD
+ - Common ancillary findings: Pericardial effusion and periaortic hematoma (↑ risk of rupture)
+ - Common features of IMH (dissection variant)
+ - Intramural blood pool (IBP)
+ - a.k.a. branch pseudoaneurysms
+ - Pool of contrast with no obvious or very small luminal communication (1-2 mm)
+ - Communications with aortic branches (e.g., intercostal arteries); MIP reformations helpful to show communications
+ - Usually not associated with worse prognosis [unlike ulcer-like projection (ULP)]
+ - ~ 50% resolve on follow-up; risk of incomplete regression: Larger size &/or visible connection to intercostal/lumbar arteries
+ - ULP
+ - Term "ulcer-like projection" is (catheter) angiographic term, describing focal projection of contrast beyond expected contour; it is purely descriptive, and can be contrast filling PAU, intimal tear, traumatic lesion, or even normal infundibular origin of, e.g., intercostal branch; in setting of IMH/dissection variant, it is used to describe contrast within IMH though > 3 mm wide intimal defect
+ - Contrast outpouching with wide (> 3 mm) connection to flow lumen
+ - New intimal injury in area of high shear stress w/o atherosclerosis
+ - Descending aorta > ascending aorta > arch (like PAU)
+ - Usually not noted at initial CTA; can develop in ~ 1/3 of patients within 1-4 months
+ - Implies poor prognosis and unfavorable outcome (↑ risk of evolution to dissection, rupture or, often saccular aneurysmal dilation)
+ - Highest risk: ULP > 20-mm diameter, > 15-mm depth, or in ascending aorta/arch
+ - Differentiation from penetrating aortic ulcer (PAU): Lack of irregular intima/atherosclerosis, no IMH, not seen initially; can be indistinguishable
+ - PAU: Intramural blood can be associated with acute PAU
+ - Intimal disease in severe atherosclerosis; ulcer-like plaque burrows through intima into media
+ - May be associated with focal or segmental IMH
+ - Results in wall thickening; smooth outer bulge related to vessel remodeling
+ - PAU + IMH > 20-mm max diameter, > 10-mm max depth, aneurysm, or increasing size on follow-up: ↑ risk of progression → consider repair
+ - Limited intimal tear (LIT): Intramural blood can be associated with LIT
+ - Outpouching of contrast typically along ascending thoracic aorta, distal to origin of left coronary artery
+ - Absence of visible intimomedial flap
+- ## MR Findings
+
+
+ - ### T1WI
+
+
+ - Acute: Isointense; subacute: Hyperintense
+ - ### T2WI
+
+
+ - Acute: Hyperintense; subacute: Lower intensity
+ - ### T1WI C+
+
+
+ - Lack of enhancement
+ - ### MRA
+
+
+ - Efficiently shows PAU, ULP, and IBP
+ - Phase-contrast MR
+ - Lack of flow unless coexistent AD
+- ## Echocardiographic Findings
+
+
+ - ### Echocardiogram
+
+
+ - Transesophageal echocardiography is equivalent to CT and MR in aortic root but limited elsewhere
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - CT most cost effective and fastest to perform
+ - ### Protocol advice
+
+
+ - Always include thin-slice NECT and CTA
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Aortitis (Takayasu and Giant Cell Arteritis)
+
+
+ - Inflammation of large and medium-sized arteries
+ - Scattered areas of stenosis ± aneurysm
+ - Parietal thickening simulates IMH on CTA
+ - Not hyperdense on NECT
+ - Mural enhancement on MR or delayed CT with contrast
+- ## IgG4 and Erdheim-Chester Disease Aortitis or Periaortitis
+
+
+ - Circumferential enhancing aortic or periaortic soft tissue
+ - Hyperintense on NECT
+ - Focal or diffuse
+ - Aneurysmal dilation or lumen stenosis less common
+ - IgG4 > ECD
+ - Infiltrating periaortic soft tissue
+ - Also perirenal infiltration in ECD
+- ## Aortic Aneurysm With Mural Thrombus
+
+
+ - Mural thrombus in lumen of dilated aorta and not in wall
+- [Aortic Dissection](/document/aortic-dissection/57e3428e-1f18-4f38-95c6-f7fe2d93c00a)
+ - Clinically, IMH presentation may be identical to AD or LIT
+ - AD with thrombosed false lumen may simulate IMH on CTA
+ - Thrombosed false lumen is typically not hyperdense on NECT; AD is also irreversible
+ - LIT with subadventitial hematoma may be indistinguishable from IMH
+ - LIT often lacks ↓ luminal aortic diameter, may have periaortic fluid, and may exhibit discrete external aortic bulge (aortic stretch mark)
+- ## Aortic Sarcoma
+
+
+ - Extremely rare
+ - Difficult to differentiate from exophytic atherosclerotic plaque
+ - Noncalcified discrete aortic wall mass
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Medial degeneration with isolated intimal tear (small, usually undetectable by CT), contained hemorrhage in vessel media, no exit tear
+ - Causes: Spontaneous vasa vasorum rupture, microscopic intimal tears, PAU, thrombosed false lumen of AD, trauma
+ - It is now accepted that communications with medial hematoma do exist
+ - ### Associated abnormalities
+
+
+ - May be associated with thoracic or abdominal aortic aneurysm
+ - Development of ULP, PAU, IBP; can enlarge, progress to frank AD, rupture
+- ## Staging, Grading, & Classification
+
+
+ - Stanford classification
+ - Type A (~ 40%): Ascending aorta ± descending aorta
+ - Type B (~ 60%): Excludes ascending aorta
+- ## Gross Pathologic & Surgical Features
+
+
+ - PAU
+ - Ulcerated atherosclerotic plaque
+ - May progress to aortic rupture
+ - IMH
+ - Represent intimal defect too small on CT but nearly always seen at surgery or gross pathology
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Abrupt onset of severe chest or back pain
+ - Hypertension
+ - 10-30% of all acute aortic syndromes
+ - Presentation can be indistinguishable from AD and LIT
+ - ### Other signs/symptoms
+
+
+ - Diaphoresis, hypotension, and tachycardia
+- ## Demographics
+
+
+ - ### Age
+
+
+ - 7th decade
+ - ### Sex
+
+
+ - M = F
+ - ### Epidemiology
+
+
+ - Prevalence of Marfan syndrome/connective tissue disorders not as high as AD
+- ## Natural History & Prognosis
+
+
+ - Evolution patterns of IMH
+ - Spontaneous resolution (~ 10%)
+ - Evolution or coexistence with AD (28-47%)
+ - Aortic rupture (20-45%)
+ - Stability over time (rare)
+ - Predictors of poorer prognosis and ↑ mortality
+ - Dilated aorta: ascending 48-55 mm, descending 40-41 mm
+ - IMH thickness > 10-11 mm
+ - PAU in acute phase: ↑ incidence of progression
+ - Especially when PAU > 20 mm
+ - Stanford type A
+ - Mortality of type A IMH with only medical treatment is ~ 40%
+ - IBP: Not considered at ↑ risk for disease progression
+ - Often regresses over time
+- ## Treatment
+
+
+ - Similar to that for typical AD
+ - Type A IMH: Surgical treatment
+ - Asian factor: Good results reported with initial medical management for type A IMH in Asian countries
+ - Type B IMH: Medical treatment and close follow-up
+ - Endovascular or open surgery reserved for complications
+ - PAU should be treated early (e.g., endovascular or surgery)
+ - IBP usually observed and follow-up CTA
+
+ 1dc2c8f0-8031-44c2-a1b9-8665ab78da55
+
+## References
+
+# Selected References
+
+1. [Steinbrecher KL et al: CT of the difficult acute aortic syndrome. Radiographics. 42(1):69-86, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34951836%5Bpmid%5D)
+1. [Ko JP et al: Chest CT angiography for acute aortic pathologic conditions: pearls and pitfalls. Radiographics. 41(2):399-424, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33646903%5Bpmid%5D)
+1. [Murillo H et al: Aortic dissection and other acute aortic syndromes: diagnostic imaging findings from acute to chronic longitudinal progression. Radiographics. 41(2):425-46, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33646901%5Bpmid%5D)
+1. [Moral S et al: Clinical implications of focal intimal disruption in patients with type B intramural hematoma. J Am Coll Cardiol. 69(1):28-39, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28057247%5Bpmid%5D)
+1. [Sailer AM et al: Prognostic significance of early aortic remodeling in acute uncomplicated type B aortic dissection and intramural hematoma. J Thorac Cardiovasc Surg. 154(4):1192-200, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28668458%5Bpmid%5D)
+1. [Gutschow SE et al: Emerging concepts in intramural hematoma imaging. Radiographics. 36(3):660-74, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27163587%5Bpmid%5D)
+1. [Valente T et al: MDCT distinguishing features of focal aortic projections (FAP) in acute clinical settings. Radiol Med. 120(1):50-72, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25249411%5Bpmid%5D)
+1. [Akin I et al: Penetrating aortic ulcer, intramural hematoma, acute aortic syndrome: when to do what. J Cardiovasc Surg (Torino). 53(1 Suppl 1):83-90, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22433727%5Bpmid%5D)
+1. [Chin AS et al: State-of-the-art computed tomography angiography of acute aortic syndrome. Semin Ultrasound CT MR. 33(3):222-34, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22624967%5Bpmid%5D)
+1. [Harris KM et al: Acute aortic intramural hematoma: an analysis from the International Registry of Acute Aortic Dissection. Circulation. 126(11 Suppl 1):S91-6, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22965999%5Bpmid%5D)
+1. [Kitai T et al: Detection of intimal defect by 64-row multidetector computed tomography in patients with acute aortic intramural hematoma. Circulation. 124(11 Suppl):S174-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21911809%5Bpmid%5D)
+1. [Wu MT et al: Intramural blood pools accompanying aortic intramural hematoma: CT appearance and natural course. Radiology. 258(3):705-13, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21212368%5Bpmid%5D)
+1. [Vilacosta I et al: Acute aortic syndrome: a new look at an old conundrum. Postgrad Med J. 86(1011):52-61, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20065341%5Bpmid%5D)
+1. [Fleischmann D et al: Acute aortic syndromes: new insights from electrocardiographically gated computed tomography. Semin Thorac Cardiovasc Surg. 20(4):340-7, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19251175%5Bpmid%5D)
+1. [Williams DM et al: Aortic branch artery pseudoaneurysms accompanying aortic dissection. Part II. Distinction from penetrating atherosclerotic ulcers. J Vasc Interv Radiol. 17(5):773-81, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16687742%5Bpmid%5D)
+1. [Wu MT et al: Images in cardiovascular medicine. Multislice computed tomography of aortic intramural hematoma with progressive intercostal artery tears: the Chinese ring-sword sign. Circulation. 111(5):e92-3, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15699269%5Bpmid%5D)
+1. [Demers P et al: Stent-graft repair of penetrating atherosclerotic ulcers in the descending thoracic aorta: mid-term results. Ann Thorac Surg. 77(1):81-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14726040%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Aortic Intramural Abnormality
+DDX:75d7b37f-bc37-493b-8961-8b2a9001fb94
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial NECT in a patient with Stanford type A intramural hematoma (IMH) shows crescent-shaped wall hyperdensity at the ascending and descending aorta.*
+
+
+*Axial NECT in a patient with Stanford type A intramural hematoma (IMH) shows crescent-shaped wall hyperdensity at the ascending and descending aorta.*
+
+
+*Axial CT angiography shows crescentic aortic wall thickening in the ascending and descending aorta. Type A IMH is typically treated surgically.*
+
+
+*Narrow window settings can improve the visibility of the hyperattenuated, crescent-shaped ascending and descending aortic wall. A hypoattenuating, crescent-shaped thickened intima is also notable due to atherosclerosis.*
+
+
+*The orange overlay represents the hyperattenuating IMH. The light green overlay marks the thickened, atherosclerotic intima.*
+
+
+*Axial black-blood MR in a patient with type B IMH shows the presence of hyperintense crescentic IMH along the descending thoracic aorta. This intensity behavior is consistent with acute hemorrhage.*
+
+
+*Axial SSFP MR in the same patient shows that the IMH is iso- to slightly hyperintense when compared with adjacent muscles. MR is as efficient as CT in determining and characterizing the presence of IMH and may be used when CT is unavailable or contraindicated.*
+
+
+*Sagittal oblique CTA shows type B IMH . Note decreased diameter of the aortic lumen along the area of IMH. This is a helpful finding to differentiate from aortitis, which usually does not exhibit such features.*
+
+
+*Axial chest CTA in a patient with IMH shows a large penetrating aortic ulcer (PAU) with a broad neck along the distal thoracic aorta, likely the primary cause of the IMH. Note also an ulcer-like projection (ULP) along the aortic arch.*
+
+
+*Axial CECT in a patient with type B IMH shows focal aortic projection consistent with ULP. This collection was not present on the baseline CTA but developed on follow-up imaging.*
+
+
+*Axial CECT in the same patient shows interval enlargement of ULP and new aortic dissection . ULP are characterized by absence on baseline, development on follow-up imaging, and wide communication with the aortic lumen. ULP implies a poorer prognosis with evolution into aortic dissection or aneurysm.*
+
+
+*Axial MIP CTA in a patient with type B IMH shows an intramural blood pool (IBP) . There is no clear communication with the aortic lumen, but there is visible communication with an adjacent intercostal artery .*
+
+
+*Sagittal oblique CTA in the same patient shows multilevel IBP related to contiguous intercostal arteries. This appearance is often referred to as Chinese ring-sword sign. Overall, IBP (as opposed to ULP) can be closely observed with follow-up imaging (i.e., CTA), as they will typically resolve.*
+
+
+*Axial chest NECT in a patient with type B IMH treated clinically shows the classic crescentic aortic wall hyperdensity . CTA demonstrates the classic crescentic wall thickening .*
+
+
+*Axial NECT and CTA in the same patient show complete resolution of the IMH. IMH can resolve, remain stable, or progress to a variety of complications, including aortic dissection and rupture. CT remains the best follow-up tool for patients with type B IMH.*
+
+
+*Axial chest NECT and CTA in a patient with type A IMH show a coexistent descending thoracic aortic dissection .*
+
+
+*Axial chest NECT and CTA in a patient with incomplete dissection show crescentic hyperdensity and thickening along the ascending aorta, findings identical to those seen in IMH. Note the discrete bulging along the posterior ascending thoracic aorta, distal to the origin of the left coronary artery. This characteristic finding is classically seen in incomplete aortic dissection.*
+
+
+### Additional Images
+
+
+*Axial chest NECT in a patient with chest pain and type A IMH shows crescentic hyperdensity , consistent with IMH along the ascending and descending thoracic aorta.*
+
+
+*Axial chest CTA in the same patient shows concentric thickening along the ascending and descending thoracic aorta as well as a small IBP along the descending thoracic aorta . Note the small pericardial and bilateral pleural effusions, which are common nonspecific findings.*
+
+
+*Sagittal oblique CTA in the same patient shows IMH extending from the distal aortic arch into the descending thoracic aorta. Note the mild aortic luminal narrowing of the affected area when compared with the normal descending aorta. This latter feature may be helpful when differentiating IMH from incomplete dissection with subadventitial hemorrhage.*
+
+
+*Axial NECT in a patient with Erdheim-Chester disease and mild chest pain shows that the circumferential aortic wall thickening is hyperattenuating, measuring 60 HU, and could mimic an intramural hematoma.*
+
+
+*Sagittal oblique CTA in the same patient shows long-segment, continuous circumferential thickening of the entire thoracic and superior abdominal aorta . The pleura is also thickened. There is no aneurysm or stenosis. ECD can mimic IMH on imaging.*
+
+
+*Axial NECT shows circumferential high attenuation in the ascending aorta with an area of more dense calcification .*
+
+
+*Axial CECT shows the circumferential ascending aortic soft tissue . The imaging finding were called an IMH on CT. The patient was taken for surgery and a Bentall procedure was performed. Pathology showed IgG4 sclerosing periaortitis. There was no IMH.*
+
diff --git a/docs_md/articles/cardiac-calcifications_c53ad786-4464-4a04-a3e9-ccd286e1f8fc.md b/docs_md/articles/cardiac-calcifications_c53ad786-4464-4a04-a3e9-ccd286e1f8fc.md
new file mode 100644
index 0000000..283dbe3
--- /dev/null
+++ b/docs_md/articles/cardiac-calcifications_c53ad786-4464-4a04-a3e9-ccd286e1f8fc.md
@@ -0,0 +1,137 @@
+---
+title: "Cardiac Calcifications"
+docid: "c53ad786-4464-4a04-a3e9-ccd286e1f8fc"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Cardiac Calcifications"
+ slug: "cardiac-calcifications"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "76a1cfbf-a803-4de9-be9e-02798943ac36"
+imageCount: 9
+lastUpdated: "03/17/22"
+pageDescription: "Cardiac Calcifications"
+pageKeywords: "Cardiac, Differential Diagnosis, Cardiac Calcifications"
+pageTitle: "Cardiac Calcifications | STATdx"
+enhancedTitle: "Cardiac Calcifications"
+type: "DDX"
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Cardiac Calcifications"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Most common pitfall is misidentifying which anatomic structure is calcified
+ - Cardiac calcifications more common in dialysis patients
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Coronary Artery**
+ - Curvilinear, parallel lines most commonly in proximal coronary arteries and at vessel branch points
+ - Amount of calcium correlates with amount of coronary plaque but not degree of stenosis
+ - Presence correlates with risk of future cardiac events
+ - **Mitral Valve**
+ - Annular calcifications
+ - Associated with mitral valve insufficiency
+ - Valvular calcifications
+ - Suggests stenosis, most often due to rheumatic heart disease
+ - **Aortic Valve**
+ - Calcification burden correlates with stenosis severity
+ - Bicuspid valve
+ - Young patients
+ - Coexistent coarctation
+ - Degenerative
+ - Patients > 60 years of age
+ - Risk factor for coronary atherosclerosis
+ - Rheumatic heart disease
+ - Patients > 35 years of age
+ - Coexistent mitral valve stenosis
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Pericardial**
+ - Associated with constrictive pericarditis
+ - **Myocardial**
+ - Indicates prior infarction; myocardial fat will likely be present
+ - **Other Cardiac Valves and Chambers**
+ - Tricuspid valve
+ - Most commonly due to rheumatic heart disease; mitral and aortic valve will likely be calcified
+ - Pulmonary valve
+ - Most commonly due to congenital pulmonary stenosis
+ - Atrial calcifications
+ - Associated with severe atrial dilation
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Mass**
+ - Chronic thrombus
+ - Atrial appendage or adjacent to infarcted myocardium
+ - Metastasis
+ - History of primary tumor
+ - Atrial myxoma
+ - Look for characteristic location and attachment
+
+
+## Images
+
+
+### Selected Images
+
+
+**Coronary Arteries**
+*Axial oblique CECT MIP shows discrete calcifications in a linear arrangement in a patient with LAD atherosclerosis. Note the presence of noncalcified plaque .*
+
+
+**Coronary Arteries**
+*Axial oblique CECT MIP shows discrete calcifications in a linear arrangement in a patient with LAD atherosclerosis. Note the presence of noncalcified plaque .*
+
+
+**Mitral Valve Leaflets**
+*Frontal radiograph shows characteristic reversed C-shaped calcification indicating mitral valve annular calcification. This calcification pattern is very common and, unlike leaflet calcification, not usually associated with stenosis.*
+
+
+**Mitral Valve Leaflets**
+*Axial NECT shows mitral valve leaflet calcifications in a patient with mitral stenosis presumed to be due to rheumatic heart disease. Note the enlarged left atrium and left atrial calcifications . The patient also has aortic stenosis and calcifications .*
+
+
+**Aortic Valve**
+*Double oblique CECT MIP shows dense calcifications of the aortic valve cusps in a patient with severe aortic stenosis. Calcium burden correlates with severity of stenosis.*
+
+
+**Pericardial**
+*Axial NECT shows pericardial calcification at the atrioventricular grooves. Note the epicardial fat to differentiate from coronary calcium.*
+
+
+**Myocardial**
+*Left ventricular outflow view shows apical calcification and wall thinning in a patient with prior myocardial infarction. Note the epicardial fat to differentiate from pericardium . Wall motion abnormality was present (not shown).*
+
+
+**Neoplastic Mass**
+*Axial NECT (left) and bright blood MR (right) show a new calcification in the RV of a 40-year-old patient with remote history of pulmonary embolus. This calcification corresponded to the presence of a chronic thrombus .*
+
+
+**Neoplastic Mass**
+*Lateral radiograph shows curvilinear calcification in the left atrium in a patient with left atrial myxoma.*
+
+
+### Additional Images
+
+
+**Mitral Valve Leaflets**
+*Four-chamber CECT shows both papillary muscle and mitral valve annular calcification. Papillary muscle calcification is commonly seen in dialysis patients.*
+
diff --git a/docs_md/articles/cardiac-mass_fa1f894a-4619-4657-b99b-a967a3e4e871.md b/docs_md/articles/cardiac-mass_fa1f894a-4619-4657-b99b-a967a3e4e871.md
new file mode 100644
index 0000000..52557c4
--- /dev/null
+++ b/docs_md/articles/cardiac-mass_fa1f894a-4619-4657-b99b-a967a3e4e871.md
@@ -0,0 +1,203 @@
+---
+title: "Cardiac Mass"
+docid: "fa1f894a-4619-4657-b99b-a967a3e4e871"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Cardiac Mass"
+ slug: "cardiac-mass"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "de88549a-e500-4b4c-8157-a415679d7913"
+imageCount: 15
+lastUpdated: "03/17/22"
+pageDescription: "Cardiac Mass"
+pageKeywords: "Cardiac, Differential Diagnosis, Cardiac Mass"
+pageTitle: "Cardiac Mass | STATdx"
+enhancedTitle: "Cardiac Mass"
+type: "DDX"
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Cardiac Mass"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Clinical impact is most affected by determination of possible malignancy
+ - Etiology of cardiac masses often cannot be distinguished with imaging
+ - Mass prevalence, coupled with ancillary findings and clinical history, is best tool in generating focused differential
+ - Thrombus is most common cardiac mass
+ - Thrombus usually is associated with causes of flow disturbance (atrial fibrillation, ventricular aneurysm, cardiomyopathy, etc.)
+ - Malignant:benign ratio = 60:1
+ - Metastasis:primary cardiac tumor ratio = 40:1
+ - Primary benign:primary malignant ratio = 3:1
+ - Primary cardiac neoplasm prevalence reported at 1 per 3,000 to 100,000 in autopsy series
+ - Malignant vs. benign
+ - Heterogeneous MR signal is nonspecific and can be seen in benign or malignant neoplasms
+ - Most lesions are T2 hyperintense and T1 isointense to myocardium
+ - Malignant tumors more often have moderate to strong enhancement than benign masses
+ - Multichamber involvement or extension into adjacent structures suggest malignant mass
+ - Myxomas (benign) are usually heterogeneous
+ - Pleural or pericardial effusion suggests primary cardiac malignancy or metastasis
+ - In absence of effusion, primary malignancy is less common and metastasis is very uncommon
+ - Right heart mass suggests metastasis
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Thrombus**
+ - MR signal characteristics vary based on age of thrombus
+ - Chronic thrombus: T1 and T2 hypointense
+ - Acute thrombus: T1 and T2 hyperintense
+ - Thrombus will not enhance on postcontrast images; best determined on subtraction postcontrast images
+ - Enhancement with vessel expansion suggests tumor thrombus
+ - Thrombus will remain dark on delayed enhancement images using long inversion time (500 ms) due to T2* shortening
+ - Signal intensity will decrease when employing gradient echo sequences vs. spin echo due to T2* shortening
+ - Commonly occur adjacent to area of heart wall hypokinesis or wall thinning
+ - Commonly occur in atrial appendages
+ - Associated with history of myocardial infarction or atrial fibrillation
+ - Polypoid thrombi more likely to embolize than smooth peripheral thrombi
+ - **Cardiac Metastases**
+ - In adults, most commonly lung, breast, lymphoma, esophagus, and melanoma primary
+ - In children, most commonly leukemia, lymphoma, neuroblastoma, Wilms, hepatoblastoma, and sarcoma
+ - ~ 90% are clinically silent
+ - Autopsy series of cancer patients showed prevalence of ~ 7%
+ - Imaging features are variable; diagnosis suggested by history of above malignancies
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Myxoma**
+ - Frequency: LA vs. RA ~ 4:1; bilateral (4%), RV (8%)
+ - 10% of cases due to autosomal dominant inheritance
+ - ~ 50% will prolapse across AV valve
+ - Many cases cause pseudo-mitral valve disease
+ - ~ 15% with calcification, more commonly seen in RA myxomas
+ - Lobulated:smooth contour ratio ~ 3:1
+ - **Sinus of Valsalva**
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Sarcoma**
+ - Most patients are symptomatic, complaining of dyspnea at time of diagnosis
+ - Most patients present with metastasis
+ - Angiosarcoma most common pathology at 33%
+ - Angiosarcoma most commonly in right atrium
+ - Other sarcoma histologies preferentially intracavitary in left atrium
+ - Commonly occur between 3rd and 5th decades
+ - Lesion morphology is variable ranging from infiltrative to endocardial
+ - Intense heterogeneous contrast enhancement
+ - Heterogeneous, mostly intermediate T1 signal and heterogeneous, mostly high T2 signal
+ - **Rhabdomyoma**
+ - Most common benign tumor in pediatric population
+ - High T2 signal and intermediate T1 signal
+ - Multiple lesions are often present
+ - Myocardial/intramural location
+ - 50% of patients have coexistent tuberous sclerosis
+ - **Fibroma**
+ - 2nd most common benign tumor in pediatric population
+ - Focal bulge, most commonly in ventricular wall, extending toward cardiac lumen
+ - Involved myocardium is hypokinetic
+ - Myocardial/intramural location
+ - Solitary
+ - Calcification common
+ - T1 iso- or hyperintense compared to myocardium
+ - T2 hypointense compared to myocardium
+ - MR and CT contrast enhancement similar to myocardium or nodular peripheral enhancement
+ - Present in 10-15% of patients with Gorlin syndrome
+ - Autosomal dominant disease with propensity to develop multiple neoplasms, such as basal cell cancers and medulloblastomas
+ - **Hemangioma**
+ - Patients usually asymptomatic
+ - Heterogeneous attenuation on unenhanced CT
+ - Hyperenhancement on enhanced CT
+ - T1 isointense compared to myocardium
+ - T2 hyperintense compared to myocardium
+ - Isointense to blood pool on balanced steady-state free precession
+ - **Lipoma**
+ - Macroscopic fat with capsule
+ - Multiple lipomas seen in tuberous sclerosis
+
+
+## Images
+
+
+### Selected Images
+
+
+**Thrombus**
+*Axial CECT shows a nonenhancing filling defect in the left ventricular apex with adjacent calcifications and wall thinning. This patient had a prior LAD territory myocardial infarct and apical hypokinesis.*
+
+
+**Thrombus**
+*Axial CECT shows a nonenhancing filling defect in the left ventricular apex with adjacent calcifications and wall thinning. This patient had a prior LAD territory myocardial infarct and apical hypokinesis.*
+
+
+**Thrombus**
+*Axial CECT shows a well-marginated filling defect in the left atrial appendage in a patient with atrial fibrillation. Atrial appendages are common locations for thrombi.*
+
+
+**Thrombus**
+*Filling defect is seen in the inferior right atrium in a young patient with testicular cancer. Although any malignancy can metastasize to the heart, this is not commonly reported for this histology. This intracardiac mass resolved after anticoagulation.*
+
+
+**Thrombus**
+*Four-chamber delayed-phase CECT using an inversion time of 500 ms in the same patient shows low signal of the filling defect at the level of the coronary sinus.*
+
+
+**Cardiac Metastases**
+*Frontal radiograph in a patient with known metastatic melanoma shows deviation of the left heart border (new compared to 1 month prior). Further imaging showed cardiac metastasis.*
+
+
+**Cardiac Metastases**
+*Axial CECT in the same patient with metastatic melanoma shows diffuse hepatic metastasis and expansion of the anterior wall of the left ventricle . Note heterogeneous contrast attenuation.*
+
+
+**Cardiac Metastases**
+*Axial CECT shows a heterogeneous mass in right and left atrium . Although sparing of the fossa ovalis suggests lipomatous hypertrophy of intraatrial septum, heterogeneous enhancement, soft tissue attenuation, and involvement of both atria indicate malignancy.*
+
+
+**Cardiac Metastases**
+*Short-axis T1 C+ MR shows an enhancing mass in the left atrium, which represented metastatic B-cell lymphoma.*
+
+
+**Myxoma**
+*Four-chamber bright-blood MR shows filling defect in LA . Mass was mobile and appeared tethered to intraatrial septum, presumably by a thin stalk.*
+
+
+**Sarcoma**
+*Four-chamber black-blood MR without (left) and with (right) fat suppression shows lipomatous hypertrophy of intraatrial septum . Note sparing of fossa ovalis and near-complete loss of signal with fat suppression . This is benign but may be confused with a cardiac mass.*
+
+
+**Sarcoma**
+*Axial CECT shows a heterogeneous, enhancing mass filling the right atrium with extension into the pericardium and obliteration of the epicardial fat . There was no pericardial effusion. Pathology revealed an angiosarcoma.*
+
+
+**Sarcoma**
+*Axial C+ MR in the same patient shows heterogeneous contrast enhancement . The right atrium is the most common location for cardiac angiosarcoma.*
+
+
+**Sarcoma**
+*Axial CECT shows left posterior atrial wall thickening with a lobulated contour . The mass has a broad attachment base. Resection demonstrated a leiomyosarcoma.*
+
+
+**Hemangioma**
+*Axial T2 black-blood MR shows a high-signal right atrial mass . Note the heterogeneous enhancement following IV contrast administration. Surgical removal revealed hemangioma.*
+
+
+### Additional Images
+
+
+**Cardiac Metastases**
+*Axial CECT shows filling defect in SVC , which was suspected to be thrombus. Multiple other filling defects were present in the right atrium (not shown). Right skin lesion is noted, and biopsy showed large B-cell lymphoma. SVC mass was later proven to be a tumor.*
+
diff --git a/docs_md/articles/chronic-posttraumatic-pseudoaneurysm_21837987-efb6-4218-90ff-22362f61a21d.md b/docs_md/articles/chronic-posttraumatic-pseudoaneurysm_21837987-efb6-4218-90ff-22362f61a21d.md
new file mode 100644
index 0000000..300fba2
--- /dev/null
+++ b/docs_md/articles/chronic-posttraumatic-pseudoaneurysm_21837987-efb6-4218-90ff-22362f61a21d.md
@@ -0,0 +1,370 @@
+---
+title: "Chronic Posttraumatic Pseudoaneurysm"
+docid: "21837987-efb6-4218-90ff-22362f61a21d"
+authors:
+ - key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
+ value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
+ - key: "5de0df07-7b3e-4678-8767-1519e1153f29"
+ value: "Dominik Fleischmann, MD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Chronic Posttraumatic Pseudoaneurysm"
+ slug: "chronic-posttraumatic-pseudoaneury-"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "adfdcbb9-fedc-497a-b70c-a523b2e278e3"
+imageCount: 23
+lastUpdated: "12/19/24"
+pageDescription: "Chronic Posttraumatic Pseudoaneurysm"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Chronic Posttraumatic Pseudoaneurysm"
+pageTitle: "Chronic Posttraumatic Pseudoaneurysm | STATdx"
+enhancedTitle: "Chronic Posttraumatic Pseudoaneurysm"
+type: "DX"
+references: true
+cases: 1
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Chronic Posttraumatic Pseudoaneurysm"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Traumatic disruption of aortic wall that goes undiagnosed in acute setting
+ - Chronic traumatic aortic injury (CTAI)
+- ## Imaging
+
+
+ - Radiography
+ - AP window mass
+ - Curvilinear calcification typically lining caudad portion of aortic arch
+ - Rightward tracheal deviation
+ - CTA
+ - Saccular dilatation at isthmus arising from inferior aspect of aortic arch
+ - Curvilinear mural calcification along saccular dilatation
+ - Ancillary findings of remote trauma
+ - Healed rib, clavicular or scapular fractures
+ - Thoracic vertebral body wedge fractures
+- ## Top Differential Diagnoses
+
+
+ - Nontraumatic aortic aneurysm
+ - In atherosclerosis, calcification often lines superoexternal portion of aortic arch and other locations
+ - Mycotic aneurysm often lacks calcifications
+ - Penetrating aortic ulcer is not common at isthmus; often with extensive atherosclerosis
+ - Mediastinal mass
+ - Ductus aneurysm
+ - Often indistinguishable from CTAI on imaging
+- ## Clinical Issues
+
+
+ - Asymptomatic; incidental finding on imaging
+ - Unknown incidence
+ - 1/3 of CTAI may rupture and cause death if untreated
+ - Preferred treatment: Endovascular repair, if anatomically suitable (often complex with arch involvement, may need branched device)
+ - Alternative treatment: Open surgical repair.
+
+# TERMINOLOGY
+
+- ## Synonyms
+
+
+ - Chronic traumatic aortic injury (CTAI)
+ - Late or unsuspected posttraumatic pseudoaneurysm
+- ## Definitions
+
+
+ - Traumatic disruption of aortic wall
+ - Not containing 3 vascular wall layers
+ - Contained by adventitia or thrombus and fibrous tissue
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Saccular aneurysm with wall calcification at level of aortic isthmus
+ - ### Location
+
+
+ - Near aortic isthmus, typical location of acute traumatic aortic injury; e.g., at undersurface of distal aortic arch, or proximal descending thoracic aorta
+ - ### Morphology
+
+
+ - Saccular, acute margins with aorta
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - CTA
+ - ### Protocol advice
+
+
+ - Use of multiplanar reformations on CTA or MRA may be helpful; 3D processing for treatment planning (TEVAR)
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - Frontal projection
+ - AP window mass
+ - Curvilinear calcification typically lining distal portion aortic arch/proximal descending aorta
+ - Rightward tracheal deviation
+ - Lateral projection
+ - Curvilinear calcified convexity (mass) at aortic isthmus
+- ## CT Findings
+
+
+ - ### CTA
+
+
+ - Saccular dilatation near aortic isthmus
+ - Acute margins with aorta, narrow ostium
+ - Curvilinear mural calcification at saccular dilatation
+ - May contain low-density thrombus
+ - May cause extrinsic compression of left main bronchus
+ - Remainder of aorta may be normal
+ - Ancillary findings of remote trauma
+ - Healed rib, clavicular or scapular fractures
+ - Thoracic vertebral body wedge fractures
+ - Traumatic diaphragmatic hernia
+- ## MR Findings
+
+
+ - ### MRA
+
+
+ - Contrast-filled saccular dilatation at aortic isthmus in continuity with aorta
+ - Intraluminal thrombus appears hypointense
+ - Black-blood and bright-blood (e.g., SSFP) are as accurate as CTA
+ - Used when CTA is contraindicated
+- ## Angiographic Findings
+
+
+ - Rarely required (often part of endovascular treatment)
+ - Angiography lacks visualization of surrounding extraaortic tissues
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Nontraumatic Aortic Pseudoaneurysm
+
+
+ - May be secondary to infection (i.e., mycotic), atherosclerosis/penetrating aortic ulcer (PAU), surgery
+ - In atherosclerosis, calcification often lines superoexternal portion of aortic arch and is also found in other locations
+ - Mycotic aneurysm often lacks calcifications
+ - PAU is uncommon at isthmus and often has extensive atherosclerosis
+ - There is history of remote trauma in CTAI, and calcifications are limited to saccular dilatation
+ - May be impossible to differentiate from pseudoaneurysm [i.e., acute traumatic aortic injury (ATAI) or CTAI] on imaging
+- ## Ductus Aneurysm
+
+
+ - May be difficult to distinguish from CTAI on imaging
+ - Smooth obtuse margins, wide ostium
+- ## Mediastinal Mass
+
+
+ - e.g., lung cancer, bronchogenic cyst
+ - CT with contrast is often diagnostic
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Posttraumatic
+ - ### Associated abnormalities
+
+
+ - Osseous fractures (rib, clavicle, sternum, thoracic spine)
+ - Rib
+ - Clavicle
+ - Sternum
+ - Thoracic spine
+ - Diaphragmatic hernia
+- ## Gross Pathologic & Surgical Features
+
+
+ - Pseudoaneurysm
+ - Not containing 3 vascular wall layers
+ - Contained by adventitia or thrombus and fibrous tissue
+ - Mural calcifications
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Asymptomatic; incidental finding on imaging
+ - Chest pain, dysphagia, dyspnea, cough, hoarseness
+ - Symptoms
+ - Chest pain
+ - Dysphagia
+ - Dyspnea
+ - Cough
+ - Hoarseness (recurrent laryngeal nerve irritation); a.k.a. Ortner syndrome
+ - Hemoptysis (aortoesophageal fistula)
+- ## Demographics
+
+
+ - ### Epidemiology
+
+
+ - Unknown incidence
+ - Majority of patients with ATAI die at scene
+ - Majority of patients with ATAI who reach hospital are treated acutely
+ - Small minority of ATAI cases remain undiagnosed and may become CTAI
+- ## Natural History & Prognosis
+
+
+ - 1/3 of CTAI rupture and cause death if untreated
+ - May rupture even years after acute injury
+ - Other complications
+ - Aortopulmonary fistula, aortoesophageal fistula
+ - Bacterial endocarditis
+ - 10-year survival rate: < 70% without surgery, > 85% with surgery
+- ## Treatment
+
+
+ - Small, asymptomatic aneurysms > 2 years after trauma can followed with CT imaging surveillance
+ - Traditional treatment: Open surgical repair
+ - Alternative treatment: Endovascular repair, if anatomically suitable
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - CTAI in patients with isthmic saccular dilatation
+
+ 2345f13c-48fe-40cb-89cd-4397aaf1c9ee
+
+## References
+
+# Selected References
+
+1. [Authors/Task Force Members et al: EACTS/STS guidelines for diagnosing and treating acute and chronic syndromes of the aortic organ. Ann Thorac Surg. 118(1):5-115, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38416090%5Bpmid%5D)
+1. [Recicarova S et al: Comprehensive multi-modality treatment of thoracic aorta pseudoaneurysms: a single-center experience. Gen Thorac Cardiovasc Surg. 72(6):387-94, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38001300%5Bpmid%5D)
+1. [Karangelis D et al: Late in-hospital rupture of a chronic post-traumatic pseudoaneurysm. Heart Views. 19(4):146-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=31057708%5Bpmid%5D)
+1. [Abed H et al: Very late rupture of a post-traumatic abdominal aortic pseudoaneurysm. BMJ Case Rep. 2017, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28130287%5Bpmid%5D)
+1. [Mesolella M et al: Delayed recurrent nerve paralysis following post-traumatic aortic pseudoaneurysm. Open Med (Wars). 11(1):215-9, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=28352797%5Bpmid%5D)
+1. [Nizet C et al: Chronic false aneurysm after a healed rupture of the aortic isthmus: TEVAR, hybrid surgery, or open arch repair? Ann Vasc Surg. 31:205.e11-6, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26631770%5Bpmid%5D)
+1. [Iddriss A et al: Chronic traumatic thoracic aortic aneurysm: 40-year follow-up. J Card Surg. 30(7):586-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25973650%5Bpmid%5D)
+1. [Pozek I et al: Chronic posttraumatic pseudoaneurysm of the thoracic aorta. Curr Probl Diagn Radiol. 41(4):126-7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22607925%5Bpmid%5D)
+1. [Marcu CB et al: Unsuspected chronic traumatic aortic pseudoaneurysm--what to do about it. Late post-traumatic aortic pseudoaneurysm. Can J Cardiol. 24(2):143-4, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18273489%5Bpmid%5D)
+1. [Katsumata T et al: Operation for chronic traumatic aortic aneurysm: when and how? Ann Thorac Surg. 66(3):774-8, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9768929%5Bpmid%5D)
+1. [Bacharach JM et al: Chronic traumatic thoracic aneurysm: report of two cases with the question of timing for surgical intervention. J Vasc Surg. 17(4):780-3, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8464102%5Bpmid%5D)
+1. [Heystraten FM et al: Chronic posttraumatic aneurysm of the thoracic aorta: surgically correctable occult threat. AJR Am J Roentgenol. 146(2):303-8, 1986](http://www.ncbi.nlm.nih.gov/pubmed/?term=3484580%5Bpmid%5D)
+1. [Finkelmeier BA et al: Chronic traumatic thoracic aneurysm. Influence of operative treatment on natural history: an analysis of reported cases, 1950-1980. J Thorac Cardiovasc Surg. 84(2):257-66, 1982](http://www.ncbi.nlm.nih.gov/pubmed/?term=7098511%5Bpmid%5D)
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '2cd036e9-d3d3-4b44-93f1-c0f8bf83ac5e', 'value': None}, {'key': 'd69a0534-8989-4b83-b19d-c3b640f22e53', 'value': 'Megan R. Saettele, MD'}, {'key': '2e78a4ff-418e-46f9-93e4-c6d95d960e35', 'value': 'Brandt C. Wible, MD'}], 'caseVersionId': '35400fe6-4d8d-474c-a7e9-d4a4f87253ba', 'description': "CTA axial images (#1-2) show a peripherally calcified outpouching (arrows) arising just distal to the left subclavian take-off. Note that there is no mass effect on adjacent structures. CTA reconstructions (#3-4) again show a large saccular peripherally calcified outpouching arising in the region of the aortic isthmus (arrows) consistent with a pseudoaneurysm. Given this patient's history of prior motor vehicle collision, this most likely represents post-traumatic pseudoaneurysm. The patient was also found to have an incidental simple liver cyst (open arrow, #3).\n\nComment: Post-traumatic aortic pseudoaneurysms are commonly found as incidental findings on radiographs or computed tomography examinations. Patients may be asymptomatic at the time of diagnosis. This is not a benign finding however, and there is a 30% mortality rate within the first 10 years without surgical or endovascular repair. Endovascular therapy offers a less invasive alternative to surgery, as well as an option for patients who are not surgical candidates.", 'history': 'Patient with a remote history of motor vehicle collision who presented to the emergency room with cough and shortness of breath; initial chest radiograph displayed AP window fullness and a subsequent CT was ordered to evaluate, as well as rule out PE. ', 'imagePoolId': 'f6bafd5f-46b6-44f7-a757-3991862a4ee4', 'name': 'Saccular aortic pseudoaneurysm', 'teachingPoint': None, 'demographics': '54 Years old male'}, {'authors': [{'key': '2cd036e9-d3d3-4b44-93f1-c0f8bf83ac5e', 'value': None}, {'key': 'd69a0534-8989-4b83-b19d-c3b640f22e53', 'value': 'Megan R. Saettele, MD'}, {'key': '2e78a4ff-418e-46f9-93e4-c6d95d960e35', 'value': 'Brandt C. Wible, MD'}], 'caseVersionId': '4bebe581-ec8b-469c-b557-e67b410544fe', 'description': 'A single axial unenhanced CT image of the chest (#1) demonstrates a large hematoma (arrows) and metallic fragments (open arrows) in the infraclavicular region. The patient was taken emergently to the angiography suite. An aortic angiogram with a pigtail catheter in the ascending aorta (#2) demonstrates a short-segment irregularity involving the left subclavian artery just distal to the vertebral origin (arrow). An aneurysm of the right brachiocephalic artery was incidentally noted (curved arrow). Images from selective left subclavian angiograms (#3-4) demonstrate a 2 cm pseudoaneurysm involving the left subclavian artery (arrows). Subsequent treatment of the injured subclavian artery was performed with a covered stent-graft. Post-deployment left subclavian angiographic images (#5-6) show the covered stent-graft to be widely patent and well positioned. There is no evidence of contrast extravasation, this finding consistent with successful exclusion of the pseudoaneurysm (arrows). Note the widely patent vertebral artery (open arrow, #6) following stent placement.\n\nComment: As opposed to a true aneurysm, which contains all three vascular wall layers, a pseudoaneurysm is contained only by adventitia or thrombus/fibrous tissue. Subclavian pseudoaneurysms most frequently result from complications of subclavian vein cannulation; blunt trauma is a less common etiology. Prior to the advent of endovascular treatments, treatment of this entity typically required resection or bypass of the aneurysm, often requiring sternotomy secondary to the vessel’s intra-thoracic course. Endovascular stenting represents a safe, minimally invasive alternative.', 'history': 'Patient presented status post gunshot trauma.', 'imagePoolId': '012e0805-f4f3-44e8-a86a-9d05b2e4515a', 'name': 'Subclavian artery pseudoaneurysm', 'teachingPoint': None, 'demographics': '42 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*PA radiograph of the chest in a young asymptomatic patient with chronic traumatic aortic injury (CTAI) and right diaphragmatic hernia shows curvilinear calcification lining the caudad part of the aortic arch . There is elevation of the right hemidiaphragm form chronic undiagnosed right diaphragmatic hernia . Note remote rib fractures .*
+
+
+*PA radiograph of the chest in a young asymptomatic patient with chronic traumatic aortic injury (CTAI) and right diaphragmatic hernia shows curvilinear calcification lining the caudad part of the aortic arch . There is elevation of the right hemidiaphragm form chronic undiagnosed right diaphragmatic hernia . Note remote rib fractures .*
+
+
+*Lateral radiograph of the chest in the same patient shows aortic bulging with intrinsic mural calcification at the isthmus.*
+
+
+*Axial chest CTA in the same patient demonstrates saccular isthmic aortic dilatation with some mural calcifications in continuity with the aortic lumen.*
+
+
+*Axial chest CTA in the same patient reveals extensive calcifications along the wall of the pseudoaneurysm. This constitutes the most common imaging appearance of CTAI. The presence of other stigmata of trauma is often helpful to differentiate from ductus aneurysm.*
+
+
+*Oblique sagittal chest CTA in an asymptomatic patient with CTAI and right diaphragmatic hernia shows a well-defined aortic pseudoaneurysm at the aortic isthmus. Note characteristic sudden change in caliber of the aorta distally, a common finding.*
+
+
+*Coronal chest CTA in the same patient demonstrates characteristic right diaphragmatic rupture with the hourglass sign of the liver and a frank hemidiaphragmatic defect .*
+
+
+*Posterior sagittal chest 3D reformation in the same patient demonstrates the aortic pseudoaneurysm and its relationship with the pulmonary artery and the left atrium. 3D reformations may be helpful for better anatomic understanding and appropriate surgical planning.*
+
+
+*PA chest radiograph in an asymptomatic patient with CTAI and a pseudoaneurysm shows curvilinear calcifications along the inferior aspect of the aortic arch.*
+
+
+*Axial chest CTA in the same patient demonstrates well-marginated saccular dilatation of the aorta at the level of the isthmus.*
+
+
+*Oblique sagittal reformation chest CTA in the same patient shows an isthmic pseudoaneurysm with intrinsic curvilinear wall calcifications. A ductus aneurysm can be difficult to differentiate from a CTAI on imaging. However, they both have similar clinical and prognostic considerations as well as treatment.*
+
+
+*PA chest radiograph in an asymptomatic patient with CTAI shows mild widening of the mediastinum.*
+
+
+*Lateral chest radiograph in the same patient demonstrates that, given a lack of significant amount of wall calcification, the abnormality (i.e., the pseudoaneurysm) is difficult to appreciate on chest radiography. While surgery has traditionally been the preferred treatment for CTAI, conservative treatment may be used in asymptomatic individuals with densely calcified pseudoaneurysms.*
+
+
+*Axial NECT in the same patient reveals contour abnormality at the level of the aortic isthmus.*
+
+
+*Axial CTA in the same patient shows a saccular aneurysm at the level of the aortic isthmus. In general, some clues that support the diagnosis include a positive clinical history of significant trauma, lack of atherosclerotic changes elsewhere, location of abnormalities at the level of the aortic isthmus, and stigmata of trauma (e.g., healed fractures, diaphragmatic hernia, etc.).*
+
+
+*Oblique CTA candy cane reformations in the same patient make the identification of the saccular aneurysm easier. The lack of mediastinal hemorrhage and other associated injuries support the chronicity of the finding.*
+
+
+*Oblique sagittal DSA in a patient with CTAI shows contrast filling the saccular outpouching . The CT and angiographic features, along with the patient's history, are consistent with a posttraumatic thoracic aortic pseudoaneurysm.*
+
+
+### Additional Images
+
+
+*Sagittal volume-rendered CTA shows a posttraumatic pseudoaneurysm with eccentric peripheral calcification .*
+
+
+*Left anterior oblique aortogram in the same patient shows contrast filling the aortic pseudoaneurysm . The location of the pseudoaneurysm allowed for successful endovascular treatment with an aortic stent graft.*
+
+
+*Sagittal CECT in a patient with prior trauma shows a large saccular pseudoaneurysm along the aortic isthmus with peripheral rim calcification , consistent with a posttraumatic pseudoaneurysm.*
+
+
+*Sagittal CECT in the same patient shows that the stent graft extends from immediately distal to the left subclavian arterial origin to the descending aorta . The pseudoaneurysm is thrombosed , and there is no endoleak.*
+
+
+*Axial CECT of the same patient shows a thrombosed saccular pseudoaneurysm sac that has been excluded by an endovascular aortic stent graft used for treatment. The endovascular graft lumen is widely patent .*
+
+
+*Axial CECT shows a peripherally calcified enhancing saccular outpouching from the distal aortic arch with compression of the left main stem bronchus . Patients may develop clinical symptoms, such as dyspnea, from compression of adjacent structures by a pseudoaneurysm.*
+
+
+*Sagittal DSA in the same patient shows contrast filling the saccular outpouching . The CT and angiographic features, along with the patient's history, are consistent with a posttraumatic thoracic aortic pseudoaneurysm.*
+
diff --git a/docs_md/articles/coarctation-of-aorta_c0b23d8c-05e3-4373-b5d9-2de1590414a7.md b/docs_md/articles/coarctation-of-aorta_c0b23d8c-05e3-4373-b5d9-2de1590414a7.md
new file mode 100644
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+++ b/docs_md/articles/coarctation-of-aorta_c0b23d8c-05e3-4373-b5d9-2de1590414a7.md
@@ -0,0 +1,582 @@
+---
+title: "Coarctation of Aorta"
+docid: "c0b23d8c-05e3-4373-b5d9-2de1590414a7"
+authors:
+ - key: "ee6ece9d-ad74-458c-a8df-11628ae7f879"
+ value: "Arzu Canan, MD"
+ - key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
+ value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Coarctation of Aorta"
+ slug: "coarctation-of-aorta"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "f12d8ff7-c299-4ea8-a15c-fc127aa71522"
+imageCount: 32
+lastUpdated: "01/23/25"
+pageDescription: "Coarctation of Aorta"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Coarctation of Aorta"
+pageTitle: "Coarctation of Aorta | STATdx"
+enhancedTitle: "Coarctation of Aorta"
+type: "DX"
+references: true
+ddx: true
+cases: 2
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Coarctation of Aorta"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Congenital narrowing of aorta, most commonly occurring just distal to left subclavian artery origin
+- ## Imaging
+
+
+ - Chest radiograph: Inferior rib notching, figure 3 sign
+ - Esophagram: Reverse figure 3 sign
+ - CTA: Focal, shelf-like narrowing of posterior/lateral aorta just distal to left subclavian origin
+ - MR
+ - Contrast-enhanced 3D MR angiography (MRA) for vessel morphology and depiction of enlarged collateral arteries
+ - Velocity-encoded cine is used to estimate pressure gradients and flow volumes
+ - Angiography
+ - Morphology of coarctation and collateral vessels
+ - Measurement of pressure gradients
+- ## Top Differential Diagnoses
+
+
+ - Pseudocoarctation
+ - Takayasu arteritis
+ - Interrupted aortic arch
+- ## Pathology
+
+
+ - Associations
+ - Bicuspid aortic valve, ventricular septal defect, patent ductus arteriosus
+ - Turner syndrome
+- ## Clinical Issues
+
+
+ - Surgical correction used for infants
+ - Balloon angioplasty used for children and adults
+ - Stent placement typically for recoarctation
+- ## Diagnostic Checklist
+
+
+ - Search for subtle signs of coarctation in any young patient with hypertension
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - **Coarctation of aorta****(CoA**, **coarc)**
+- ## Definitions
+
+
+ - Congenital narrowing of aorta, most commonly occurring just distal to left subclavian artery origin
+ - Atypical coarctation: Not involving isthmus (usually abdominal aorta)
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Location
+
+
+ - May occur anywhere in aorta or at multiple sites
+ - Preductal, ductal, or post ductal
+ - ### Size
+
+
+ - Longer segment stenosis referred to as tubular hypoplasia
+ - Focal or diffuse
+- ## Radiographic Findings
+
+
+ - Radiography
+ - **Inferior rib notching (Roesler sign)**
+ - Related to enlargement of intercostal arteries serving as collaterals
+ - Rare before 5 years of age
+ - Affects ribs 3-8; ribs 1 and 2 are not affected, as they arise from costocervical trunk and do not anastomose with distal aorta
+ - May regress post repair
+ - Unilateral rib notching may indicate aberrant subclavian artery
+ - **Figure 3 sign in up to 50% of cases**
+ - Dilated left subclavian artery produces proximal convexity
+ - Indentation at coarctation
+ - Poststenotic descending aorta produces distal convexity
+ - **Ill-defined or obscured aortic arch**
+ - Mediastinal widening
+ - Heart: Rounded apex from left ventricular hypertrophy
+ - May exhibit bicuspid aortic valve
+ - Esophagram
+ - Reverse figure 3 sign
+ - Compression on esophagus from dilated left subclavian artery and poststenotic dilatation of descending aorta
+- ## CT Findings
+
+
+ - ### CTA
+
+
+ - Excellent morphologic characterization
+ - Defines location and severity of stenosis
+ - Focal, shelf-like narrowing of posterior/lateral aorta just distal to left subclavian origin
+ - Enlarged collateral arteries indicate hemodynamic significant obstruction at coarctation site
+ - Gradient cannot be calculated
+ - Multiplanar reformations (sagittal oblique plane) and 3D volume-rendered images
+ - Useful for pre- and postoperative stent repair
+- ## MR Findings
+
+
+ - Allows morphologic and functional assessment
+ - Morphology
+ - Achieved with several MR protocols: HASTE, SSFP (TrueFISP, FIESTA), contrast-enhanced MR angiography (MRA)
+ - HASTE (dark blood) and SSFP (bright blood)
+ - Single-shot sequences; can be axial, sagittal, or coronal
+ - ECG-gated technique; can be free breathing
+ - Helpful to determine associated cardiac anomalies
+ - "Gothic" or angulated aortic morphology after coarctation surgery is associated with high risk of arterial hypertension
+ - Useful to assess for complications: Aneurysm, pseudoaneurysm, recoarctation
+ - **Contrast**-**enhanced MRA**
+ - 3D acquisition on oblique sagittal axis (i.e., "candy cane" axis) after intravenous contrast
+ - Better results with ECG-gated and breath-holding techniques
+ - Planimetry: Determine orthogonal diameters and areas from proximal to distal to ductus
+ - **Indexed minimal aortic cross-sectional area (cm²/m²)**
+ - Adjusted to body surface area
+ - Best predictor of severity
+ - < 0.33 cm²/m² indicates severe coarctation (gradient ≥ 20 mmHg)
+ - Demonstrates enlarged collateral arteries
+ - **Velocity-encoded cine MR**
+ - Flow-sensitive phase-contrast technique, segmented acquisition
+ - Requires breath-holding and ECG-gating techniques
+ - **Heart rate-corrected deceleration time (sec⁻⁰****·****⁵)**
+ - Adjusted to heart rate
+ - Flow value at cessation of flow - peak flow rate/time interval from peak to cessation
+ - Deceleration time = [(flow at end of deceleration) - (peak systolic flow)]/(deceleration time)
+ - Adjustment to heart rate with Bazett formula: 1/(R-R interval)⁰·⁵
+ - Excellent predictor of severity
+ - ≥ 0.30 sec⁻⁰·⁵ indicates severe coarctation (gradient ≥ 20 mmHg)
+ - Amount of collateral flow
+ - Enlarged collateral vessels are reliable indicator of hemodynamic significance
+ - Retrograde blood flow in relevant mediastinal or intercostal arteries is indicative of collateral flow
+ - Percentage increase in flow between aorta immediately distal to coarctation and just above diaphragmatic crura due to collateral flow
+ - Normal subjects: 7% ± 6% decrease
+ - Coarctation (gradient < 20 mmHg): No increase
+ - Coarctation (gradient ≥ 20 mmHg): 83% ± 50% mean increase
+ - Pressure gradient (ΔP)
+ - Calculated with peak velocity (v)
+ - Modified Bernoulli equation (short-segment stenoses only): ΔP = 4v²
+ - Gradient ≥ 20 mmHg indicates severe coarctation
+ - May be used to quantify aortic valve stenosis and regurgitation
+ - **Combination of indexed minimal cross-sectional area and heart rate-corrected deceleration time best****predicts hemodynamically significant coarctation**
+ - Cine MR
+ - Cine SSFP or GRE (segmented acquisition)
+ - Requires breath-holding and ECG-gating techniques
+ - Gold standard to assess left ventricular hypertrophy (myocardial thickness and mass)
+ - Enables characterization of bicuspid aortic valve
+ - Allows identification of turbulent and jet flows that may indicate stenosis or regurgitation
+ - 4D flow MR
+ - Calculates collateral blood flow
+ - Vortical flow pattern in regions of poststenotic dilatation
+ - Helical flow pattern may be seen in angulated postsurgical aorta
+- ## Echocardiographic Findings
+
+
+ - ### Echocardiogram
+
+
+ - Suprasternal long-axis view
+ - Color Doppler estimates gradient across coarctation
+ - Classic findings: Narrowing of isthmus and posterior indentation or shelf
+- ## Angiographic Findings
+
+
+ - Vessel morphology and direct measurement of pressure gradient (ΔP)
+ - < 20 mmHg: Mild coarctation
+ - ≥ 20 mmHg: Suggests need for intervention
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - MR and MRA often fully characterize and determine needs of treatment
+ - Angiography remains gold standard; used when MR is inconclusive
+ - ### Protocol advice
+
+
+ - MR to include sagittal oblique plane through aortic arch and perpendicular plane through coarctation for measurement of cross-sectional diameter
+
+# DIFFERENTIAL DIAGNOSIS
+
+- [Pseudocoarctation](/document/pseudocoarctation/a5a7c623-d5cb-4bb1-b628-986d9ca1f94a)
+ - Older adult with elongation and kinking of aorta related to atherosclerosis
+ - No hemodynamically significant stenosis or collateral vessels
+- [Takayasu Arteritis](/document/takayasu-arteritis/3b589c7b-d975-4f2c-a5b1-ff83dd856ee7)
+ - Inflammatory narrowing of unknown etiology
+ - Narrowing &/or occlusion of aorta and branch vessels, rarely isolated to aortic isthmus
+- ## Interrupted Aortic Arch
+
+
+ - Complete absence of continuity between 2 segments of aorta
+ - Nearly always manifests in neonates
+- [Traumatic Pseudoaneurysm](/document/lung-trauma/f78f6d72-3ed4-4836-ab68-34b8e7698f71)
+ - History of trauma, healed rib, and other skeletal fractures
+ - Narrowing of descending thoracic aorta may coexist with pseudoaneurysm
+- ## Inferior Rib Notching Differential
+
+
+ - Neurofibromatosis
+ - Venous collaterals (superior vena cava obstruction)
+ - Decreased pulmonary blood flow (tetralogy of Fallot, pulmonary atresia)
+ - Blalock-Taussig shunt (ribs 1 and 2)
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Abnormal development of embryologic left 4th and 6th aortic arches
+ - Muscular theory
+ - Migration of tissue from ductus arteriosus into aortic wall and subsequent contraction
+ - Hemodynamic theory
+ - Decreased aortic blood flow during fetal development may not allow proper aortic growth
+ - Increased incidence of coarctation in disorders in which left ventricular outflow tract obstruction reduces aortic blood flow
+ - Decreased incidence of coarctation in disorders in which decreased ductal flow is present (e.g., tetralogy of Fallot)
+ - ### Genetics
+
+
+ - Association with Turner syndrome (up to 20% of patients have coarctation)
+ - ### Associated abnormalities
+
+
+ - **Bicuspid aortic valve (reported in 50-85%)**
+ - Ventricular septal defect
+ - Patent ductus arteriosus
+ - Shone syndrome: Aortic coarctation, subaortic stenosis, parachute mitral valve, supravalvular mitral membrane
+ - Cerebral aneurysms (2.5-10.0%)
+ - Variable evidence regarding increased risk of coronary artery disease
+- ## Staging, Grading, & Classification
+
+
+ - Classification (controversial)
+ - Previously used classifications (e.g., infantile and adult type) discouraged due to overlapping manifestations
+ - Pathophysiologic classification
+ - Preductal: Stenosis proximal to ductus arteriosus
+ - Distal blood flow relies on patency of ductus; closure can be life threatening
+ - 5% of all children with Turner syndrome
+ - Ductal: At insertion of ductus arteriosus
+ - Clinically evident after ductus closure
+ - Post ductal: Stenosis distal to ductus arteriosus
+ - **Simple coarctation**
+ - Occurs in isolation
+ - Often localized just beyond left subclavian artery origin (post ductal)
+ - **Complex coarctation**
+ - Occurs in presence of other intracardiac anomalies, thus, tends to manifest in infancy
+ - Often preductal
+- ## Gross Pathologic & Surgical Features
+
+
+ - Obstructing membrane or ridge of tissue near aortic isthmus
+ - May develop cystic medial necrosis adjacent to coarctation site; predisposes to aneurysm or dissection
+ - 3 main collateral pathways
+ - Subclavian artery → internal mammary artery → intercostal arteries
+ - Subclavian artery → thyrocervical and costocervical trunks → thoracoacromial and descending scapular arteries → intercostal arteries
+ - Subclavian artery → vertebral artery → anterior spinal artery → intercostal arteries
+- ## Microscopic Features
+
+
+ - Loss of smooth muscle cells and cystic medial necrosis when associated with bicuspid aortic valve
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Presentation depends on degree of stenosis and associated abnormalities
+ - **Neonates**
+ - Asymptomatic if coarctation not severe or patent ductus arteriosus
+ - If severe coarctation or closed ductus arteriosus, may have heart failure
+ - Decreased femoral pulses, associated murmurs
+ - **Children****and****adults**
+ - May be asymptomatic
+ - Leg claudication
+ - Differential blood pressure between upper and lower extremities, diminished femoral pulses
+ - Angina pectoris
+ - Severe hypertension
+ - Often leads to chronic heart failure
+ - Intracranial aneurysm ± subarachnoid bleeding
+ - Stroke
+ - Murmur associated with bicuspid aortic valve
+ - Epistaxis
+ - ### Other signs/symptoms
+
+
+ - Turner syndrome: Short, webbed neck; broad chest; pigmented facial nevi; short 4th metacarpals
+- ## Demographics
+
+
+ - ### Age
+
+
+ - Related to degree of narrowing and presence of associated abnormalities
+ - ### Sex
+
+
+ - M:F = 2:1
+ - ### Ethnicity
+
+
+ - White:Asian = 7:1
+ - ### Epidemiology
+
+
+ - Incidence: 2-6 per 10,000 births
+ - Comprises 5-10% of cases of congenital heart disease
+ - Represents 7% of all critically ill infants with congenital heart disease
+- ## Natural History & Prognosis
+
+
+ - Without repair
+ - Average age of death: 35-42 years
+ - 75% mortality rate by age 46
+ - Due to aortic dissection or rupture, heart failure, myocardial infarct, and cerebral hemorrhage
+ - With repair
+ - ~ 90% survival rate at 20 years; decreased chance of survival with increased age at repair
+ - Recoarctation (2-14%)
+ - Associated with younger age at surgery
+ - Postoperative aneurysms (increased risk after patch aortoplasty)
+ - Long-term survival rate decreased due to hypertension, coronary artery disease, dissection
+ - Pregnancy-related issues
+ - Untreated coarctation: Increased risk of dissection and intracranial hemorrhage
+ - Treated coarctation: Increased rate of miscarriage and preeclampsia
+ - All patients require lifelong follow-up with cardiologist trained in congenital heart disease
+- ## Treatment
+
+
+ - Indications for treatment
+ - Infant with severe stenosis and heart failure
+ - Longstanding hypertension
+ - Hemodynamically significant stenosis (gradient > 20 mmHg)
+ - Extensive collateral flow
+ - Female patient planning pregnancy
+ - Surgical correction: 1st-line treatment for infants
+ - Resection with end-to-end anastomosis
+ - Higher risk of spinal artery injury and restenosis
+ - Left subclavian flap aortoplasty
+ - Sacrifice left subclavian artery and vertebral artery (to avoid subclavian steal)
+ - Bypass graft
+ - Used if area of narrowing is too long for end-to-end repair
+ - Prosthetic patch or interposition graft
+ - Rarely used due to long-term risk of infection or aneurysm with prosthetic material
+ - Acute complications
+ - Surgical mortality rare
+ - Paradoxical hypertension, recoarctation, hypertension, paraplegia due to spinal artery damage, recurrent laryngeal or phrenic nerve injury, subclavian steal
+ - Postcoarctectomy syndrome
+ - Abdominal pain that may progress to intestinal wall hemorrhage or even perforation
+ - Late complications
+ - Aortic aneurysm, recurrent coarctation, hypertension
+ - Balloon angioplasty
+ - 1st-line treatment for older children and adults for native coarctation or recoarctation
+ - Not recommended for infants due to increased rate of recurrence
+ - Acute complications (rare)
+ - Dissection, stroke
+ - Late complications
+ - Recoarctation, aneurysm, endocarditis, hypertension
+ - Stent placement
+ - Generally reserved for recoarctation
+ - Complications
+ - Acute rupture, dissection, stent fracture or migration
+ - Aneurysm in up to 11%
+ - More long-term data needed
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - Search for subtle signs of coarctation in any young patient with hypertension
+- ## Image Interpretation Pearls
+
+
+ - Enlarged collaterals imply significant stenosis
+
+ 32fcf972-f7c9-4372-aa4a-24d0fc34829f
+
+## References
+
+# Selected References
+
+1. [Chetan D et al: Challenges in diagnosis and management of coarctation of the aorta. Curr Opin Cardiol. 37(1):115-22, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34857719%5Bpmid%5D)
+1. [Saran N et al: Management of coarctation and aortic arch anomalies in the adult. Semin Thorac Cardiovasc Surg. 33(4):1061-8, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34091017%5Bpmid%5D)
+1. [Kim YY et al: Aortic coarctation. Cardiol Clin. 38(3):337-51, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32622489%5Bpmid%5D)
+1. [Bhave NM et al: Multimodality imaging of thoracic aortic diseases in adults. JACC Cardiovasc Imaging. 11(6):902-19, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29880113%5Bpmid%5D)
+1. [Dijkema EJ et al: Diagnosis, imaging and clinical management of aortic coarctation. Heart. 103(15):1148-55, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28377475%5Bpmid%5D)
+1. [Gach P et al: Multimodality imaging of aortic coarctation: from the fetus to the adolescent. Diagn Interv Imaging. 97(5):581-90, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27132712%5Bpmid%5D)
+1. [Karaosmanoglu AD et al: CT and MRI of aortic coarctation: pre- and postsurgical findings. AJR Am J Roentgenol. 204(3):W224-33, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25714305%5Bpmid%5D)
+1. [Muzzarelli S et al: Usefulness of cardiovascular magnetic resonance imaging to predict the need for intervention in patients with coarctation of the aorta. Am J Cardiol. 109(6):861-5, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22196785%5Bpmid%5D)
+1. [Kim HK et al: Cardiovascular anomalies in Turner syndrome: spectrum, prevalence, and cardiac MRI findings in a pediatric and young adult population. AJR Am J Roentgenol. 196(2):454-60, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21257900%5Bpmid%5D)
+1. [Muzzarelli S et al: Prediction of hemodynamic severity of coarctation by magnetic resonance imaging. Am J Cardiol. 108(9):1335-40, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21861960%5Bpmid%5D)
+1. [Teo LL et al: Prevalence of associated cardiovascular abnormalities in 500 patients with aortic coarctation referred for cardiovascular magnetic resonance imaging to a tertiary center. Pediatr Cardiol. 32(8):1120-7, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21512788%5Bpmid%5D)
+1. [Hope MD et al: Clinical evaluation of aortic coarctation with 4D flow MR imaging. J Magn Reson Imaging. 31(3):711-8, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20187217%5Bpmid%5D)
+1. [Kimura-Hayama ET et al: Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 30(1):79-98, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20083587%5Bpmid%5D)
+1. [Gaca AM et al: Repair of congenital heart disease: a primer--part 2. Radiology. 248(1):44-60, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18458241%5Bpmid%5D)
+1. [Hom JJ et al: Velocity-encoded cine MR imaging in aortic coarctation: functional assessment of hemodynamic events. Radiographics. 28(2):407-16, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18349448%5Bpmid%5D)
+1. [Warnes CA et al: ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 118(23):e714-833, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18997169%5Bpmid%5D)
+1. [Ou P et al: Aortic arch shape deformation after coarctation surgery: effect on blood pressure response. J Thorac Cardiovasc Surg. 132(5):1105-11, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17059930%5Bpmid%5D)
+1. [Shih MC et al: Surgical and endovascular repair of aortic coarctation: normal findings and appearance of complications on CT angiography and MR angiography. AJR Am J Roentgenol. 187(3):W302-12, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16928909%5Bpmid%5D)
+1. [de Bono JP et al: Long term follow up of patients with repaired aortic coarctations. Heart. 91(4):537-8, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15772226%5Bpmid%5D)
+1. [Konen E et al: Coarctation of the aorta before and after correction: the role of cardiovascular MRI. AJR Am J Roentgenol. 182(5):1333-9, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15100141%5Bpmid%5D)
+1. [Lee EY et al: MDCT evaluation of thoracic aortic anomalies in pediatric patients and young adults: comparison of axial, multiplanar, and 3D images. AJR Am J Roentgenol. 182(3):777-84, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14975985%5Bpmid%5D)
+1. [Sebastià C et al: Aortic stenosis: spectrum of diseases depicted at multisection CT. Radiographics. 23 Spec No:S79-91, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14557504%5Bpmid%5D)
+1. [Steffens JC et al: Quantification of collateral blood flow in coarctation of the aorta by velocity encoded cine magnetic resonance imaging. Circulation. 90(2):937-43, 1994](http://www.ncbi.nlm.nih.gov/pubmed/?term=8044965%5Bpmid%5D)
+1. [Sloan RD et al: Coarctation of the aorta; the roentgenologic aspects of one hundred and twenty-five surgically confirmed cases. Radiology. 61(5):701-21, 1953](http://www.ncbi.nlm.nih.gov/pubmed/?term=13112418%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Left Ventricular Enlargement
+DDX:fbb972de-3e13-4c67-b7a4-f8901aa2efb8
+
+### Left Ventricular Enlargement
+DDX:f62409ca-8fb2-46b3-9919-2e1e6adf07b7
+
+### Narrowed Aorta
+DDX:763503a4-a7b8-4aff-8846-3dfbe312125c
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': 'bab06bb9-d576-4b97-ad6b-dc7e810c3367', 'description': 'Typical CT features of abnormal aortic contour from aortic coarctation.\n\nCTA (#1-5) shows enlarged systemic collateral (open arrows). Segment of descending aorta (arrows, #2,3) is either small or nearly absent. Sagittal oblique shows characteristic narrowing (arrow) from coarctation.', 'history': 'Uncontrolled hypertension.', 'imagePoolId': '793035bb-240a-46fe-913b-9cf3bf6c60c7', 'name': 'Aortic contour', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'd68ea291-9e92-4251-97bf-6397569c066b', 'description': 'Typical radiographic features of mediastinal contour from aortic coarctation.\n\nRadiographs (#1,2) show abnormal mediastinal contours (open arrows) from aortic coarctation. Cardiac contour (arrows) consistent with left ventricular hypertrophy.', 'history': 'Hypertension.', 'imagePoolId': 'cd41adb1-6ea4-4276-9a20-fffa91f8e412', 'name': 'Mediastinal contour', 'teachingPoint': None, 'demographics': '28 Years old male'}, {'authors': [{'key': 'b00d2bdb-66e1-41ed-90b4-c52904f4d598', 'value': 'Seth Kligerman, MD, MS'}], 'caseVersionId': 'd9926f5c-6b27-4c12-9606-0671843ddbb1', 'description': 'Coned-down PA radiograph (#1) shows subtle areas of rib notching (arrows), giving the undersurface of the ribs a wavy appearance. Additionally, there is rapid narrowing of the proximal descending thoracic aorta (curved arrow), which then returns to normal size more distally (open arrow). This pattern is sometimes referred to as the "figure of 3" sign. Axial images from a cardiac CT at the level of the arch (#2), the proximal descending thoracic aorta (#3) and more distal descending thoracic aorta (#4) show a normal size arch (arrow, #2) with marked focal narrowing of the proximal descending aorta (arrow, #3). Distal to this, the aorta returns to normal size (arrow, #4). Note the extensive collaterals in the chest wall and mediastinum (open arrows, #2-3). Full field of view image from a cardiac CT (#5) shows intercostal collateral arteries (arrows, #5), which cause the rib notching. Sagittal multiplanar reformat from the cardiac CT (#6) shows the location of the coarctation in the descending thoracic aorta (arrow).', 'history': 'Patient with uncontrolled hypertension and blood pressure gradient of over 30 mmHg between the left arm and left leg. ', 'imagePoolId': 'a8be3956-8383-4977-aa09-910d438d5366', 'name': 'Coarctation, aorta, post-ductal', 'teachingPoint': None, 'demographics': '37 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'dcd4c2ac-af2a-4e83-a495-9bc9c6ccc6eb', 'description': 'Typical CT and radiographic features of rib notching from aortic coarctation.\n\nCTA (#1-6) shows numerous collaterals (open arrows). Descending aorta is dilated (post-stenotic) (arrows, #2-6). Coarctation (open black arrow, #3) was less than 5 mm in diameter. Coronal reconstruction (#7) shows "figure 3 sign" (arrows). Volume reconstruction (#8) shows coarctation (arrow). Radiographs (#9,10) shows rib notching (open arrows).', 'history': 'Uncontrolled hypertension on three anti-hypertensive drugs.', 'imagePoolId': '852cb0d1-d115-4fe2-b917-7fad134c72e2', 'name': 'Rib notching', 'teachingPoint': None, 'demographics': '27 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': '4cd95bfe-e455-44d4-88a1-75b02f480c80', 'description': 'Typical radiographic and MR features of aortic narrowing from coarctation.\n\nRadiograph (#1) shows normal aortic contours. Multiple small pulmonary calcifications are probably from previous histoplasmosis. T1WI MRs (#2-3) show abrupt narrowing (arrows) of the descending aorta. Cine MRs (#4-6) show short segment aortic narrowing (open arrows) and collateral vessels (arrow, #6).', 'history': 'Hypertension.', 'imagePoolId': 'ae06339c-44dc-4d6a-90ca-a927e5de6f21', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '32 Years old male'}, {'authors': [{'key': '8e8c445a-2b2f-435d-b348-855b7921ad53', 'value': 'Christopher G. Anton, MD'}], 'caseVersionId': 'ec2164ac-e323-4ab0-bc64-a5b3d4fff986', 'description': 'Typical case of aortic coarctation on chest radiographs and angiography.\n\nRadiographs (#1, 2) show mild left ventricular prominence and post stenotic dilation of the descending aorta ("3" sign, arrow, #1). Aortic angiography (#3-5) show aortic coarctation (arrows) and post stenotic dilation (open arrows) of the descending aorta. 4 years after balloon angioplasty; radiograph (#6) and aortic angiography (#7) were performed to evaluate for re-coarctation. Radiograph (#6) again shows the "3" sign (arrow). Aortic angiography (#7) shows no re-coarctation or aneurysm with mild residual narrowing.', 'history': 'Patient with known aortic coarctation presents for precatheterization chest radiograph.', 'imagePoolId': '821f74d5-7a31-4407-8de8-0b1822c58aca', 'name': 'Balloon dilation', 'teachingPoint': None, 'demographics': '5 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '55c0cff5-49cc-4f10-af0f-9bbe4379c141', 'value': 'Howard Mann, MD'}], 'caseVersionId': 'f1ac2367-736e-42c1-a4f1-f6172213cfd3', 'description': 'Typical rib notching in aortic coarctation.\n\nRadiographs (#1-3) demonstrates notching (arrows) of the inferior surfaces of posterior left 5th (#2) and right 4th (#3) ribs.', 'history': 'Long-standing hypertension.', 'imagePoolId': '74d8582b-ac70-4185-bf5d-5e18aa0b3dad', 'name': 'Rib notching', 'teachingPoint': None}, {'authors': [{'key': 'fff9b5a0-8473-401a-8da6-d1366705ec01', 'value': 'Jeffrey P. Kanne, MD'}], 'caseVersionId': '112313c7-2488-4b82-9e5d-a034b9f2b5d9', 'description': 'PA chest radiograph (#1) shows a focal narrowing (arrow) in the proximal descending aorta. Sagittal T2 FSE MR image (#2) shows focal narrowing (arrow) in the proximal descending aorta with post-stenotic dilation (curved arrow). Sagittal T2* GRE cine MR image (#3) shows a dephasing jet (arrows) distal to the aortic narrowing.', 'history': 'Heart murmur.', 'imagePoolId': '20c69921-e2f6-47a3-ba49-2248520d9d32', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '30 Years old male'}, {'authors': [{'key': '8e8c445a-2b2f-435d-b348-855b7921ad53', 'value': 'Christopher G. Anton, MD'}], 'caseVersionId': '20d90d0d-4c24-4ecb-8eeb-d1535c0d16d3', 'description': 'Typical case of aortic coarctation with rib notching.\n\nRadiograph (#1) shows bilateral inferior rib notching. Aortic angiography (#2, 3) show coarctation (arrow, #2) of the aorta and numerous collateral vessels (white arrows, #3) with enlargement of the internal mammary arteries (black arrows, #3). Aortic angiography (#4, 5) after stent (curved arrow, #4) placement shows improvement but mild residual narrowing (arrow, #5) at the stent across the coarctation.', 'history': 'Patient with known history of coarctation and new onset upper extremity hypertension presents for precatheterization chest radiographs and angiographic stent placement.', 'imagePoolId': '71e1fdde-8064-44bd-8c03-005ce2babf0b', 'name': 'Stent, rib notching', 'teachingPoint': None, 'demographics': '16 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '55c0cff5-49cc-4f10-af0f-9bbe4379c141', 'value': 'Howard Mann, MD'}], 'caseVersionId': '87b73b7e-407c-4978-ac97-2cd88d7649a9', 'description': 'Typical radiographic and CT features of aortic narrowing from aortic coarctation.\n\nRadiographs (#1-2) show rib notching (open arrows) and abnormal aortic contour (arrows). CECT images (#3-6) show large collaterals (open arrows) and aortic narrowing (arrows, #5-6). Bone window (#7) shows rib notching (open arrows). Sagittal reconstruction (#8) shows aortic narrowing (arrow). Volume reconstruction (#9) shows aortic narrowing (arrow).', 'history': 'Hypertension.', 'imagePoolId': '762d40e6-b4b5-4a3c-85d4-5e1bce136b35', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '27 Years old male'}, {'authors': [{'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}, {'key': '3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1', 'value': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI'}], 'caseVersionId': '2b552581-b70a-4216-9502-5bd2d595bf0f', 'description': 'Sagittal VR image from CTA (#1) shows severe narrowing (arrows) of the proximal aspect of the descending aorta consistent with focal aortic coarctation. As shown in VR CTA (#1-3), there are multiple collateral arteries (open arrows) to bypass this area of aortic narrowing. As shown on axial CTA (#4), though the aortic valve has a trileaflet morphology during diastole (arrows), functional images should be reviewed to assess the aortic valve during systole given the high association between aortic coarctations and bicuspid aortic valves. On occasion, a bicuspid valve with a prominent raphe may mimic a normal trileaflet valve during diastole.', 'history': 'Patient presented with hypertension.', 'imagePoolId': '7e9a7762-a9bb-440b-9cda-d52491f07693', 'name': 'Aortic coarctation with multiple collaterals', 'teachingPoint': None, 'demographics': '35 Years old female'}, {'authors': [{'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}], 'caseVersionId': '2d64b2db-0d26-4e46-86d9-389f99a23c7d', 'description': 'VR images from cardiac CTA (#1-2) show a bicuspid aortic valve (arrows, #1); a small partially calcified raphe is also present (open arrow, #1). There is mild narrowing (curved arrows, #2) of the proximal aspect of the descending aorta from previous aortic coarctation repair.', 'history': 'Patient with history of aortic coarctation status post repair.', 'imagePoolId': '5d5824be-aac3-49a2-ac41-33cd2b35e726', 'name': 'Bicuspid aortic valve, history of aortic coarctation', 'teachingPoint': None, 'demographics': '57 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '22439123-9e23-4130-9448-b3b5a5426bf3', 'description': 'Typical CT features of aortic contour abnormality from coarctation associated with ventricular septal defect.\n\nCECT (#1-5) shows aneurysmal dilatation of proximal descending aorta (arrows). Sagittal oblique reconstructions (#6,7) shows coarctation anomaly (arrows). Ventricular septal defect (open arrow, #6). CECT (#8) shows ventricular septal defect (arrow). \n\nComment: Lack of collaterals suggests that there is no hemodynamic gradient.', 'history': 'Chest pain.', 'imagePoolId': '8f73cacd-1cd2-4b9a-8274-4a3bd9f952ce', 'name': 'Aortic aneurysm with ventricular septal defect', 'teachingPoint': None, 'demographics': '62 Years old female'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '87f05bfe-9fc3-434d-a689-0eb7ab1ea444', 'description': 'Typical CT features of coarctation of aorta.\n\nCECT (#1-4) shows extensive arterial collaterals (arrows) and aortic narrowing (open arrows, #2,3). Sagittal oblique reconstructions (#5,6) show coarctation (open arrows), dilated ascending aorta (curved arrow, #5), and aortic valve prosthesis (arrows). Coronal and sagittal reconstructions (#7,8) show dilated intercostal artery causing rib erosion (open arrows). Volume reconstructions (#9,10) shows collaterals (arrows, #9) and coarctation (open arrows).', 'history': 'Hypertension.', 'imagePoolId': 'c4b83649-38db-41a0-928a-17ac7430160c', 'name': 'Rib erosion', 'teachingPoint': None, 'demographics': '41 Years old female'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '1b61a4cf-a8fb-4e95-8e07-7e1135fd5c42', 'description': 'Typical radiographic and US features of aortic contour abnormality of coarctation of aorta.\n\nRadiographs (#1,2) shows "3" configuration of proximal descending aorta (open arrows). Doppler US (#3) of renal artery shows parvus tardus signifying upstream stenosis.', 'history': 'Hypertension.', 'imagePoolId': 'fdaee957-c09d-487d-a923-b64d85bb93d0', 'name': 'Aortic contour', 'teachingPoint': None, 'demographics': '9 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'ff62466f-4e10-4fa4-8139-53e8adb03ef8', 'description': 'Variant CT features of narrowing aorta from restenosis coarctation.\n\nCECTs (#1-5) show aortic narrowing (open arrows, #1-2, 5) and post-stenotic dilatation (arrows, #3-5). Radiographs (#6-7) show an endovascular stint (open arrows) post treatment. CECT (#8) shows bicuspid aortic valve (arrow).', 'history': 'Previous coarctation repair.', 'imagePoolId': '5ba32dba-0932-4018-a078-312902f4c64f', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '39 Years old male'}, {'authors': [{'key': '6f4e2c1e-8c5f-4fec-a244-9b7a171e7873', 'value': 'Helen T. Winer-Muram, MD'}], 'caseVersionId': '30b8348d-a052-4984-8580-0b08c93e0028', 'description': 'Variant of coarctation. Mediastinal mass represents pseudocoarctation. \n\nRadiograph (#1) shows two convexities (open arrows) at the left superior mediastinum. Radiograph (#2) shows a convexity (arrow) and concavity (open arrow) that represents kinking of an elongated thoracic aorta. CECT (#3-5) shows that there is no stenosis (open arrow) or post-stenotic dilatation (arrows) at the proximal descending aorta consistent with the diagnosis of pseudocoarctation.', 'history': 'Asymptomatic', 'imagePoolId': '0fd24734-abd2-4bf8-b138-02828ecae6ff', 'name': 'Mediastinal mass', 'teachingPoint': None, 'demographics': '73 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '71dc2a7d-41c8-4f57-bec4-928e7f92bf25', 'description': 'Variant CT features of bypass surgery for aortic coarctation.\n\nCECT (#1-3) shows bypass (open arrows) for aortic coarctation.CECT (#4) shows left ventricular hypertrophy (arrows). Sagittal oblique (#5-7) reconstructions show aortic bypass (arrows) and typical contour for coarctation (open arrows).', 'history': 'Surgery for coarctation.', 'imagePoolId': '2f402172-a927-4688-a2b7-da11ecfa3ee4', 'name': 'Surgical correction', 'teachingPoint': None, 'demographics': '42 Years old female'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'a22923d2-26d3-4288-9234-d1d653f6378a', 'description': 'Variant CT radiographic and CT features of postoperative coarctation repair aortic aneurysm and aneurysmal dilatation ascending aorta from bicuspid aortic valve.\n\nRadiographs (#1, 2) show aneurysmal dilatation (arrows) at level of aortic arch and aneurysmal dilatation of ascending aorta (open arrow, #1). CTA (#3-6) shows aneurysm (arrows, #3, 4) in region of previous coarctation repair. Aneurysm neck (open arrow, #3). Aneurysmal dilatation of ascending aorta (open arrows, #5, 6) from bicuspid aortic valve. Focal stenosis main pulmonary artery (curved arrow, #5). Sagittal oblique reconstruction (#7) shows dilated ascending aorta (open arrow) and aneurysm at level of previous coarctation repair (arrow).', 'history': 'Coarctation repair as child.', 'imagePoolId': '523eb95c-6706-41fb-a600-7f922d0d56f7', 'name': 'Postoperative aortic aneurysm', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'b2567037-3209-4a0e-8cf4-d046c452a689', 'description': 'Variant radiographic and CT features of pseudoaneurysm following aortic coarctation repair.\n\nRadiographs (#1-4) shows mediastinal contour abnormality (arrows). CECT (#5-9) shows aneurysm (arrows, #5-7, 9) at site of previous repair. Endograft has been place across the aneurysm (open arrows).', 'history': 'Previous surgical repair aortic coarctation.', 'imagePoolId': '495aa2d3-f56a-4b36-8d58-18d3f07dc939', 'name': 'Pseudoaneurysm', 'teachingPoint': None, 'demographics': '59 Years old female'}, {'authors': [{'key': 'a081f7d3-2c54-4779-846e-0d82cea35329', 'value': 'Alexander Bankier, MD'}], 'caseVersionId': 'b38e9001-0bff-4176-bc09-8d412150b98f', 'description': 'Variant CT features of aortic narrowing from aortic coarctation.\n\nBoth coronal (#1-4) and parasagittal (#5-7) reformations show the manifestations of pseudocoarctation. Narrowing of the aorta (arrow, #5,6) best seen on the parasagittal images, whereas dilatation of the supraaortic branches (open arrow, #3-6) better seen on the coronal images. Note that the descending aorta (curved arrow, #7) is of normal diameter.', 'history': 'Routine screening examination, incidental finding on echocardiography.', 'imagePoolId': 'bc685044-cc2e-44fd-ade9-51ca63fd9fdd', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '32 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': 'bff521b6-04d4-4e8f-a2c4-2a228a3c096e', 'description': 'Variant CT features of aortic contour from pseudocoarctation.\n\nNECT (#1-5) shows abnormal aortic contour (arrows) from pseudocoarctation. Note absence of collaterals. Sagittal oblique reconstruction (#6) shows notching of proximal aorta (arrow).', 'history': 'Asymptomatic', 'imagePoolId': '2afe9b1b-6942-4464-a01d-77fc985e6730', 'name': 'Pseudocoarctation', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': 'e33d0134-1101-487e-b575-87ef2ca58cf0', 'description': 'Variant CT and MR features of pseudocoarctation with bicuspid aortic valve.\n\nCECTs (#1-3) show a focal deformity of the aorta (open arrows). CECT reconstruction (#4) shows focal buckling of the proximal descending aorta (open arrow). The aorta is not narrowed and there are no collaterals. Bone window (#5) shows faint calcification (curved arrow) of the aortic valve. MR cine (#6) shows the bicuspid aortic valve (curved arrow).', 'history': 'Murmur.', 'imagePoolId': '37b37df3-b20b-43a8-9609-566ca12e4eb2', 'name': 'Pseudocoarctation', 'teachingPoint': None, 'demographics': '47 Years old female'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*PA radiograph of the chest demonstrates the classic figure 3 morphology in a patient with aortic coarctation. Note the area of stenosis , dilated subclavian artery , and poststenotic dilatation .*
+
+
+*PA radiograph of the chest demonstrates the classic figure 3 morphology in a patient with aortic coarctation. Note the area of stenosis , dilated subclavian artery , and poststenotic dilatation .*
+
+
+*Lateral radiograph of the chest in the same patient reveals an indentation along the aortic isthmus, representing the stenosis. While the figure 3 sign is not frequently seen, its presence suggests the diagnosis and should prompt additional evaluation.*
+
+
+*Axial CTA of the aorta shows a classic coarctation . Note the relatively normal caliber of the aorta proximal and distal to the critical stenosis. There are extensive mediastinal collaterals seen as serpiginous vessels around the coarctation and dilated internal mammary arteries, which also reflect collateral flow.*
+
+
+*Oblique CTA 3D reformation in the same patient shows the coarctation . Note the intercostal arteries , which appear dilated due to collateralization.*
+
+
+*PA chest radiographs in 2 different patients with aortic coarctation show an ill-defined mediastinal widening on the left and mediastinal contour abnormality on the right. Visualization of the classic figure 3 sign is often obscured by the presence of mediastinal collaterals.*
+
+
+*PA chest radiograph shows an inferior rib notching , the so-called Roesler sign, a classic radiographic sign of aortic coarctation produced by dilation of collateral vasculature. (Courtesy L. Heyneman, MD.)*
+
+
+*Axial CTA images at the prestenotic level (left) and the coarctation site (right) show characteristic focal narrowing due to the coarctation and dilated internal mammary and intercostal arteries that serve as collateral pathways.*
+
+
+*Oblique CTA MIP reformation in the same patient better delineates the coarctation , poststenotic dilatation of the descending thoracic aorta, tortuous and dilated internal mammary artery , and intercostal collateral arteries .*
+
+
+*Volume-rendered 3D CTA in a patient with aortic coarctation allows for morphologic assessment of the coarctation and provides an overall appreciation of the extent of collateralization. 3D reformations are most helpful for clinicians/surgeons to get the overall picture of the 3D configuration of the pathology.*
+
+
+*DSA in a patient undergoing angiography for subarachnoid hemorrhage shows the catheter tip proximal to an incidentally detected tight aortic coarctation.*
+
+
+*Short-axis SSFP MR through the aortic valve shows a bicuspid aortic valve in a patient with aortic coarctation. Note that the aortic valve has only 2 cusps . This is a common association in patients with aortic coarctation.*
+
+
+*Axial SSFP MR through the area of aortic coarctation shows an ascending aorta normal in diameter, a diminutive proximal descending aorta in the area of coarctation , and a relatively normal diameter of the more distal descending aorta .*
+
+
+*Sagittal SSFP MR images in a patient with aortic coarctation show a well-defined long-segment area of stenosis in the proximal descending aorta .*
+
+
+*Oblique aortic MRA MIP reformation in the same patient shows marked stenosis with extensive regional collaterals resulting from a hemodynamically significant obstruction. MRA is useful (as is CT) for determining the minimal aortic cross-sectional area and for evaluating the pressure gradients and flow volumes.*
+
+
+*Candy cane view from MRA of the thoracic aorta in a patient with repaired aortic coarctation shows mild residual narrowing at the aortic isthmus.*
+
+
+*4D-flow image of the same patient shows mild increased velocity at the repaired coarctation site, indicated in red. 4D flow images allow for quantification of flow at multiple levels, including valve levels, and also visually demonstrate flow patterns.*
+
+
+*Short-axis SSFP cine MR in this patient with coarctation shows concentric thickening of the left ventricular myocardium , consistent with left ventricular hypertrophy. This is a sequela from longstanding upper body arterial hypertension.*
+
+
+*Anterior and posterior MIP views of sagittal aortic MRA demonstrate coarctation and extensive chest wall and mediastinal collaterals . Collateral vessels are better characterized when thin-slice images (e.g., MRA) are reformatted to MIPs.*
+
+
+*This axial phase-contrast MR is from a patient with Shone complex that includes coarctation. While this sequence does not provide good morphologic correlation, it allows calculation of flow velocities and volumes over time, which may be used to quantify heart rate-corrected deceleration time.*
+
+
+*3D volume-rendered MRA in the same patient shows coarctation with poststenotic dilatation. Shone complex includes coarctation, supravalvular mitral ring, parachute mitral valve, and subaortic stenosis.*
+
+
+*This oblique sagittal CTA of the aorta is from a patient with coarctation who underwent successful endovascular stent placement after a failed treatment with angioplasty. CT and MR allow for follow-up and assessment of stent complications.*
+
+
+*Oblique sagittal CTA images in a patient with remote history of surgically corrected aortic coarctation show aneurysmatic dilatation of the left subclavian artery and the proximal descending aorta , a known complication after this type of surgery.*
+
+
+### Additional Images
+
+
+*Sagittal graphic shows high-grade, short segmental narrowing of the thoracic aorta distal to the ductus arteriosus.*
+
+
+*PA chest radiograph shows barium in the esophagus and the characteristic figure 3 sign in a patient diagnosed with aortic coarctation.*
+
+
+*PA chest radiograph (coned down to the left lung) shows left-sided rib notching at multiple levels . The patient was subsequently diagnosed with coarctation of the aorta.*
+
+
+*Sagittal oblique CECT shows short-segment, high-grade coarctation involving the proximal descending aorta . This patient had left upper extremity hypertension.*
+
+
+*Sagittal CECT of a patient with aortic coarctation shows circumscribed high-grade narrowing of the proximal descending thoracic aorta.*
+
+
+*Sagittal NECT shows high-grade stenosis of the proximal descending aorta of a 27-year-old patient with coarctation. Elongation of the supraaortic vessels is also visible.*
+
+
+*Sagittal oblique T1 C+ FS MR shows focal narrowing of the proximal descending thoracic aorta. Turbid flow is seen as hypointensity distal to the narrowing .*
+
+
+*Sagittal oblique CECT MIP of a 19-year-old man after repair of aortic coarctation shows an endovascular stent in the proximal descending aorta.*
+
+
+*Frontal radiograph of the chest in a patient with aortic coarctation is shown. Note that, despite cardiomegaly, the mediastinum does not appear widened but rather has ill-defined borders. Often, the radiographic findings are nonspecific. In this case, the hazy mediastinal borders are due to mediastinal collaterals.*
+
+
+*Oblique sagittal SSFP MR in the same patient demonstrates a marked stenosis of the proximal descending aorta in keeping with aortic coarctation.*
+
diff --git a/docs_md/articles/dilatation-of-thoracic-aorta_3b177a9c-04f3-4d15-9d18-decc5c236e7e.md b/docs_md/articles/dilatation-of-thoracic-aorta_3b177a9c-04f3-4d15-9d18-decc5c236e7e.md
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@@ -0,0 +1,145 @@
+---
+title: "Dilatation of Thoracic Aorta"
+docid: "3b177a9c-04f3-4d15-9d18-decc5c236e7e"
+authors:
+ - key: "c4c34d93-63f8-4d7e-93b2-9a7232b87ec6"
+ value: "John P. Lichtenberger, III, MD"
+breadcrumbs:
+ -
+ name: "Chest"
+ slug: "chest"
+ treeNodeId: "23b17a2b-c629-4f3b-b960-0bfdc5d138ca"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "41e2b114-bb5b-4430-86a8-4c667ef0f50a"
+ -
+ name: "Thoracic Aorta"
+ slug: "thoracic-aorta"
+ treeNodeId: "595e605a-6053-4b51-9848-2d0aed1b9299"
+ -
+ name: "General Imaging Patterns"
+ slug: "general-imaging-patterns"
+ treeNodeId: "f7c484b2-c364-43b7-ac6b-82d2f0444c3e"
+ -
+ name: "Dilatation of Thoracic Aorta"
+ slug: "dilatation-of-thoracic-aorta"
+ treeNodeId: null
+category: "Chest"
+cmeTopicId: "b38d09b5-ad4e-4ebc-b077-20db7f4c0371"
+documentVersionId: "2cc5bdb2-db16-482c-aca6-0ffc420779dd"
+imageCount: 8
+lastUpdated: "02/10/20"
+pageDescription: "Dilatation of Thoracic Aorta"
+pageKeywords: "Chest, Differential Diagnosis, Thoracic Aorta, General Imaging Patterns, Dilatation of Thoracic Aorta"
+pageTitle: "Dilatation of Thoracic Aorta | STATdx"
+enhancedTitle: "Dilatation of Thoracic Aorta"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Chest"
+ - "Differential Diagnosis"
+ - "Thoracic Aorta"
+ - "General Imaging Patterns"
+ - "Dilatation of Thoracic Aorta"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Ascending aorta dilated ≥ 4.0 cm, aneurysmal ≥ 5.0 cm
+ - Descending thoracic aorta aneurysmal ≥ 4.0 cm
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Atherosclerotic Aneurysm**
+ - Fusiform aortic aneurysms most common
+ - Typically arise from ascending aorta
+ - Dilation of aorta typically in presence of significant atherosclerotic disease
+ - Commonly contain thrombus
+ - Saccular aneurysms have increased risk of rupture
+ - **Penetrating Atherosclerotic Ulcer**
+ - Focal contrast-filled outpouching of aortic wall; underlying atherosclerotic disease
+ - More common in descending aorta or aortic arch
+ - Associated with intramural hematoma, aortic dissection
+ - Vast majority of patients have underlying hypertension and history of smoking
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Ductus Diverticulum**
+ - Focal outpouching along anterior wall at aortic isthmus
+ - Smooth angles, obtuse shoulders
+ - Sagittal images may show diverticulum contiguous with ligamentum arteriosum
+ - May contain thrombus and wall calcification
+ - **Traumatic Pseudoaneurysm**
+ - Most common location is aortic isthmus (may also be seen at aortic root or diaphragmatic hiatus)
+ - Focal contrast-filled outpouching along undersurface of aortic arch/anterior wall of aortic isthmus
+ - Unlike ductus diverticulum, traumatic pseudoaneurysms have sharp angles, irregular margins, and possible visible intimal flap
+ - Associated mediastinal hematoma may be present
+ - **Postoperative Pseudoaneurysm**
+ - Contrast-filled outpouching beyond walls of aorta associated with surgical changes
+ - Commonly seen with aortic valve replacement (especially in cases of endocarditis) and graft repair
+ - Noncontrast imaging useful to detect high-attenuation surgical material mimicking pseudoaneurysm
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Aortitis**
+ - Most commonly involve thoracic aorta: Takayasu and giant cell arteritis
+ - Leads to wall thickening, irregular aortic contours, aneurysm formation, and narrowing/occlusion
+ - Aneurysm may be fusiform or saccular
+ - Calcification may be present in chronic cases
+ - MR findings
+ - Thickened walls with high signal intensity on T2WI
+ - Enhancement of arterial wall
+ - Mycotic aortitis may cause dilation
+ - Hematogenous spread of organisms (*Staphylococcus aureus* and *Salmonella* species most common)
+
+## References
+
+# Selected References
+
+1. [Saremi F et al: Image predictors of treatment outcome after thoracic aortic dissection repair. Radiographics. 38(7):1949-72, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30312138%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Atherosclerotic Aneurysm**
+*PA chest radiograph shows dilation of the thoracic aorta, evidenced by abnormal contour of the aortic arch and displacement of the intrathoracic trachea to the right . The ascending aorta is visible overlying the right hilum.*
+
+
+**Atherosclerotic Aneurysm**
+*PA chest radiograph shows dilation of the thoracic aorta, evidenced by abnormal contour of the aortic arch and displacement of the intrathoracic trachea to the right . The ascending aorta is visible overlying the right hilum.*
+
+
+**Atherosclerotic Aneurysm**
+*Axial CECT of the same patient shows aneurysm of the descending thoracic aorta and calcified and noncalcified atherosclerotic disease of the aorta. Atherosclerosis is the most common cause of aortic dilation.*
+
+
+**Traumatic Pseudoaneurysm**
+*Double oblique MRA of the thoracic aorta in a patient with a history of thoracic trauma shows a saccular aneurysm of the aortic arch. Acute angles of the aneurysm differentiate this pseudoaneurysm from a ductus diverticulum.*
+
+
+**Traumatic Pseudoaneurysm**
+*Composite image with axial CECT (left) and NECT (right) shows focal dilation of the ascending aorta with high attenuation in the expected location of the medial aortic wall representing postsurgical material rather than aortic pathology.*
+
+
+**Postoperative Pseudoaneurysm**
+*PA chest radiograph of a patient with a history of bicuspid aortic valve and coarctation status post stent placement in the proximal descending thoracic aorta shows new dilation of the aortic arch and descending aorta . The intrathoracic trachea is displaced to the right .*
+
+
+**Postoperative Pseudoaneurysm**
+*Coronal reformatted CECT of the same patient shows the descending thoracic aortic stent complicated by large, complex contrast outpouchings , consistent with postoperative pseudoaneurysms.*
+
+
+**Aortitis**
+*Axial CECT of a patient with sepsis and chest pain shows aneurysmal dilation of the proximal descending thoracic aorta with associated diffuse aortic wall thickening and enhancement , consistent with infectious aortitis.*
+
+
+**Aortitis**
+*Fused coronal reformatted FDG PET/CT of the same patient shows diffuse FDG avidity of the thickened, aneurysmal descending thoracic aorta . A large left pleural effusion is also present. Staphylococcus and salmonella are frequent organisms causing infectious aortitis.*
+
diff --git a/docs_md/articles/dilated-aorta_9daee273-f1e9-4cf9-a979-8990a9b82e40.md b/docs_md/articles/dilated-aorta_9daee273-f1e9-4cf9-a979-8990a9b82e40.md
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+++ b/docs_md/articles/dilated-aorta_9daee273-f1e9-4cf9-a979-8990a9b82e40.md
@@ -0,0 +1,199 @@
+---
+title: "Dilated Aorta"
+docid: "9daee273-f1e9-4cf9-a979-8990a9b82e40"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Dilated Aorta"
+ slug: "dilated-aorta"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "a4834ace-8f60-4350-a339-72988d153f26"
+imageCount: 15
+lastUpdated: "03/17/22"
+pageDescription: "Dilated Aorta"
+pageKeywords: "Cardiac, Differential Diagnosis, Dilated Aorta"
+pageTitle: "Dilated Aorta | STATdx"
+enhancedTitle: "Dilated Aorta"
+type: "DDX"
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Dilated Aorta"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Pathology indicated by outer diameter measurements
+ - Measurements providing high specificity for pathology
+ - Ascending > 4.5 cm
+ - Proximal descending > 3.2 cm
+ - Ascending:descending ratio > 1.5:1
+ - Isthmus:hiatus ratio > 1.4:1
+ - Aorta should taper throughout course; focal distal diameter increase of > 50% is abnormal
+ - Morphology
+ - Saccular (false aneurysm): Dissection, mycotic, posttraumatic, penetrating atherosclerotic ulcer (PAU)
+ - Fusiform (true aneurysm): Atherosclerosis, valvular disease
+ - Location
+ - Ascending aorta: Valvular pathology, dissection, connective tissue disease, syphilis
+ - Descending aorta: Dissection, PAU, atherosclerotic, mycotic, posttraumatic
+ - Distance of aneurysm from major branch vessels determines feasibility of stent placement
+ - Tortuosity, calcification, and minimum luminal diameter of iliac arteries determine vascular access strategy
+ - Diameter of proximal and distal aneurysm determines selection of stent size
+ - Etiology of aneurysm (mycotic, inflammatory, or atherosclerotic) influences decision to treat surgically or endovascular
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Atherosclerotic**
+ - Descending aorta: Tortuous, diffuse intimal calcifications, mural thrombus, focal dilation
+ - Caused by intimal disease with fibrous replacement of underlying media
+ - Coexistent small and medium vessel atherosclerosis
+ - **Degenerative**
+ - Systemic hypertension: Leads to accelerated elastic fiber fragmentation and smooth muscle degeneration
+ - Ascending aortic dilation with relative preservation of root diameter
+ - Older patients
+ - **Aortic Stenosis**
+ - Dense calcifications of aortic valve
+ - Grade of stenosis related to valve area
+ - > 2.0 cm²: No hemodynamically significant stenosis
+ - 2-1.5 cm²: Mild stenosis
+ - 1.5-1 cm²: Moderate stenosis
+ - < 1 cm²: Severe stenosis
+ - Aortic bicuspid-related stenosis
+ - Young patient with calcified valve despite paucity of vascular calcifications elsewhere
+ - Prevalence of 1:1,000: Men more commonly affected
+ - Associated with aortic coarctation and patent ductus arteriosus
+ - Prone to dissection
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Aortic Dissection**
+ - Intimal calcifications displaced toward aortic lumen: Can be appreciated on unenhanced study
+ - False lumen expands, leading to aortic dilation
+ - Majority of patients present with systemic hypertension
+ - Intimal flap seen on enhanced CT, 3D MRA, or black-blood MR
+ - May occur in areas of prior intramural hematoma or penetrating atherosclerotic ulcer
+ - **Pseudoaneurysm**
+ - **Mycotic Aneurysm**
+ - Saccular configuration, irregular lumen, larger than PAU
+ - Adjacent abscess or inflammation
+ - More common etiology in young patients with thoracic aortic aneurysms
+ - Most commonly caused by bacterial infection (*Staphylococcus* and *Salmonella*) at site of prior aortic defect
+ - Patients will have prior history of sepsis, IV drug use, endocarditis
+ - **Penetrating Atherosclerotic Ulcer**
+ - Diffuse atherosclerotic disease present
+ - Penetration of contrast beyond expected outer aortic wall contour
+ - Adjacent inflammatory stranding and wall thickening present
+ - On MR, slow-flowing blood may make PAU appear thrombosed; phase contrast or MRA will more accurately characterize
+ - New PAU found with adjacent inflammation may indicate cause of symptoms in patients presenting with chest pain
+ - **Posttraumatic Pseudoaneurysm**
+ - History of high-energy blunt trauma
+ - Aortic contour abnormality at ligamentum arteriosum
+ - Calcifications seen in remote trauma
+ - Can less commonly occur at aortic root or hiatus
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Collagen Vascular Diseases**
+ - Takayasu/giant cell arteritis
+ - Radiographically indistinguishable; Takayasu suspected in patients < 40 years of age, giant cell suspected in patients > 40 years of age
+ - Wall thickening and enhancement present
+ - Branch vessel involvement present, classically subclavian stenosis
+ - Although most commonly causes stenosis, aneurysms can develop
+ - May also present with pulmonary artery stenoses
+ - **Connective Tissue Disease**
+ - Marfan syndrome, Ehlers-Danlos syndrome
+ - Connective tissue defect of aortic wall
+ - Annuloaortic ectasia present with ascending aorta dilation creates "tulip bulb" appearance
+ - Aortic root dilation often results in aortic regurgitation at presentation
+ - **Syphilis**
+ - Occurs in tertiary syphilis
+ - Frequency in developed world has markedly decreased
+ - Often manifest as descending aortic aneurysm although abdominal aortic aneurysm and sinus of Valsalva aneurysms occur
+ - Chronic inflammation leads to obliterative endarteritis causing ischemia of media and adventitia
+
+
+## Images
+
+
+### Selected Images
+
+
+**Atherosclerotic**
+*Frontal radiograph shows a dilated tortuous aorta with diffuse calcifications. Intimal disease further exacerbates medial degeneration by increasing wall stress and restricting blood flow.*
+
+
+**Atherosclerotic**
+*Frontal radiograph shows a dilated tortuous aorta with diffuse calcifications. Intimal disease further exacerbates medial degeneration by increasing wall stress and restricting blood flow.*
+
+
+**Atherosclerotic**
+*Axial CECT shows intimal disease with mural thrombus and intimal calcifications . This patient had a diffusely dilated and tortuous aorta.*
+
+
+**Atherosclerotic**
+*Coronal CECT shows extravasation of contrast from a dilated abdominal aorta. Note extravasated blood , which can easily be detected on unenhanced CT.*
+
+
+**Atherosclerotic**
+*Axial CECT shows dilated abdominal aorta with extensive mural thrombus . Calcifications occur when the thrombus is chronic and does not represent displaced intimal calcifications.*
+
+
+**Degenerative**
+*Lateral radiograph shows diffuse aortic calcifications in a patient with longstanding hypertension and a dilated ascending aorta.*
+
+
+**Aortic Stenosis**
+*Double oblique cine MR shows a bicuspid aortic valve in a young patient with a dilated ascending aorta. This image can be used to calculate valve area to quantify stenosis.*
+
+
+**Aortic Stenosis**
+*Left ventricular outflow CECT shows calcifications on the aortic cusps in an older patient with an ascending aortic aneurysm.*
+
+
+**Aortic Dissection**
+*Axial CECT shows ascending aortic false lumen dilation in acute dissection. Note the "bird beak" sign and "cob web" sign , which help identify the false lumen . This patient had a bicuspid valve and was treated with a modified Bentall procedure.*
+
+
+**Aortic Dissection**
+*Double oblique CECT shows dilation of the ascending aorta in a hypertensive patient presenting with anterior chest pain. Note the intimal flap . This patient was treated with emergent surgery.*
+
+
+**Mycotic Aneurysm**
+*Coronal CECT shows pseudoaneurysm in the mid descending aorta thought to be a mycotic aneurysm. Aside from this aneurysm, there was a paucity of disease throughout the remaining aorta.*
+
+
+**Penetrating Atherosclerotic Ulcer**
+*Axial black-blood MR shows an aortic wall defect that extends beyond the expected contour of the aortic lumen. High signal in this penetrating aortic ulcer is due to slow-flowing blood and not thrombosis.*
+
+
+**Penetrating Atherosclerotic Ulcer**
+*Coronal CECT shows a previously diagnosed penetrating atherosclerotic ulcer that progressed to frank rupture. Note the extravasated blood .*
+
+
+**Posttraumatic Pseudoaneurysm**
+*Volume-rendered image shows focal dilation of the aortic lumen at the level of the ligamentum arteriosum. This patient suffered a high-speed deceleration injury, presented with a traumatic pseudoaneurysm, and was treated with endovascular repair.*
+
+
+**Connective Tissue Disease**
+*Double oblique coronal left ventricular outflow view shows aortic root dilation and loss of sinotubular junction morphology in a patient with Marfan disease.*
+
+
+### Additional Images
+
+
+**Posttraumatic Pseudoaneurysm**
+*Sagittal oblique conventional angiographic view shows a traumatic pseudoaneurysm prior to treatment with endovascular stenting. This patient suffered high-speed deceleration trauma.*
+
diff --git a/docs_md/articles/double-aortic-arch_0b68477f-f05a-4e4c-a1a8-02fe29c292db.md b/docs_md/articles/double-aortic-arch_0b68477f-f05a-4e4c-a1a8-02fe29c292db.md
new file mode 100644
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--- /dev/null
+++ b/docs_md/articles/double-aortic-arch_0b68477f-f05a-4e4c-a1a8-02fe29c292db.md
@@ -0,0 +1,339 @@
+---
+title: "Double Aortic Arch"
+docid: "0b68477f-f05a-4e4c-a1a8-02fe29c292db"
+authors:
+ - key: "ee6ece9d-ad74-458c-a8df-11628ae7f879"
+ value: "Arzu Canan, MD"
+ - key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
+ value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Double Aortic Arch"
+ slug: "double-aortic-arch"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "3dc4caa1-09b8-4045-b792-da5ec170e56b"
+imageCount: 19
+lastUpdated: "03/09/25"
+pageDescription: "Double Aortic Arch"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Double Aortic Arch"
+pageTitle: "Double Aortic Arch | STATdx"
+enhancedTitle: "Double Aortic Arch"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Double Aortic Arch"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Double aortic arch (DAA)
+ - Persistent right and left aortic arches, each one giving rise to separate ipsilateral subclavian and carotid arteries
+- ## Imaging
+
+
+ - Chest radiography
+ - Frontal projection: Bilateral paratracheal opacities, bilateral tracheal indentations
+ - Lateral projection: Posterior tracheal indentation
+ - CTA
+ - Right aortic arch
+ - Larger and higher in most patients (right dominant)
+ - Left aortic arch
+ - Often smaller than right aortic arch
+ - 4-artery sign: Symmetric take-off of 4 aortic branches on axial image at thoracic inlet (2 ventral carotids and 2 dorsal subclavians)
+ - 1 descending aorta, usually contralateral to dominant arch (i.e., left)
+ - Airway CT
+ - Tracheomalacia: Tracheal collapse adjacent to vascular ring during expiration
+ - Bronchomalacia: Left main bronchus collapse adjacent to midline descending aorta during expiration
+- ## Clinical Issues
+
+
+ - Most common symptomatic vascular ring
+ - Typically manifests in neonates
+ - Children
+ - Dyspnea, often during feeding
+ - Stridor and wheezing (exacerbated by crying)
+ - Tachypnea, apnea
+ - Adults
+ - May be asymptomatic
+ - Treatment: Surgical division of smaller or atretic aortic arch and ligamentum arteriosus
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Double aortic arch (DAA)
+- ## Definitions
+
+
+ - Persistent right and left aortic arches, each one giving rise to separate ipsilateral subclavian and carotid arteries
+ - Variants
+ - Both arches patent and functioning
+ - Right arch patent, left arch atretic
+ - Difficult to differentiate from right aortic arch mirror image branching
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Chest radiography: Bilateral paratracheal opacities with concentric midtracheal narrowing
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - Frontal projection
+ - Bilateral paratracheal opacities
+ - Bilateral tracheal indentations
+ - Lateral
+ - Posterior tracheal indentation
+- ## Fluoroscopic Findings
+
+
+ - ### Esophagram
+
+
+ - Frontal projection: S-shaped, bilateral indentations on contrast-filled esophagus, right higher and larger than left
+ - Lateral view: Large posterior indentation, often oblique
+- ## CT Findings
+
+
+ - ### CTA
+
+
+ - Right aortic arch
+ - Larger in most patients (right dominant)
+ - More cephalad than left
+ - Courses behind esophagus
+ - Left aortic arch
+ - Often smaller than right aortic arch
+ - Rarely same size (codominant) or larger (left dominant)
+ - Left aortic arch atresia can be confused with right aortic arch
+ - Inferior tethering of left subclavian artery
+ - ± aortic diverticulum
+ - Posterior course of proximal head and neck vessels
+ - Focal narrowing of airway
+ - **4-artery sign**: Symmetric take-off of 4 aortic branches on axial image at thoracic inlet (2 ventral carotids and 2 dorsal subclavians)
+ - 1 descending aorta, usually contralateral to dominant arch (i.e., left)
+ - Airway CT
+ - Inspiration and expiration CT may help differentiate tracheomalacia from tracheal stenosis
+ - Tracheomalacia: Tracheal collapse adjacent to vascular ring during expiration
+ - Bronchomalacia: Left main bronchus collapse adjacent to midline descending aorta during expiration
+- ## MR Findings
+
+
+ - As accurate as CT in assessing vascular anatomy and tracheal stenosis
+ - Of value in young individuals due to lack of ionizing radiation
+ - Same findings as CT
+- ## Echocardiographic Findings
+
+
+ - ### Echocardiogram
+
+
+ - Suprasternal notch view is most helpful, showing 2 separate aortic arches, each giving rise to separate carotid and subclavian arteries
+ - Poor assessment of airway compression
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - MR and CT are equally accurate in assessing vascular and tracheal anatomy
+ - ### Protocol advice
+
+
+ - Multiplanar reformations are helpful in delineating arch anatomy and tracheal abnormalities
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Right Aortic Arch With Aberrant Left Subclavian Artery and Kommerell Diverticulum
+
+
+ - Kommerell diverticulum may mimic left aortic arch on frontal chest radiograph
+ - Tracheal indentation on lateral chest radiograph
+ - Differentiation usually requires cross-sectional imaging
+- ## Right Aortic Arch With Mirror Image Branching and Aortic Diverticulum
+
+
+ - Lack of inferior tethering of left subclavian artery
+ - Aortic diverticulum is more common in DAA with atretic left aortic arch
+ - DAA with atretic left aortic arch and right aortic arch with mirror image branching and aortic diverticulum are part of spectrum of vascular rings and have similar clinical implications
+- ## Left Pulmonary Artery Sling
+
+
+ - Anterior esophageal and posterior tracheal indentations
+ - May be associated with tracheomalacia
+- [Innominate Artery Compression Syndrome](/document/innominate-artery-compression-synd-/bc9f2319-6c01-4218-b75b-3481b2461738)
+ - Anterior tracheal indentation without esophageal compression
+- ## Mediastinal Mass
+
+
+ - Mediastinal masses can cause tracheal compression
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Persistence of right and left 4th aortic arches
+ - ### Associated abnormalities
+
+
+ - Often not associated with congenital heart disease
+ - 20% associated with congenital heart disease
+ - Tetralogy of Fallot (most common)
+ - Ventricular septal defect
+ - Aortic coarctation
+ - Patent ductus arteriosus
+ - Transposition of great arteries
+ - Truncus arteriosus
+ - Tracheobronchomalacia
+ - May cause persistent airways symptoms after surgery
+- ## Gross Pathologic & Surgical Features
+
+
+ - Tight vascular ring with tracheal and esophageal compression
+ - Dominance: Right (~ 70%) > left (~ 20%) > codominant (~ 5%)
+ - Smaller left arch may be partially atretic
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Children
+ - Dyspnea, often during feeding
+ - Stridor and wheezing (exacerbated by crying)
+ - Tachypnea, apnea
+ - Recurrent respiratory infections
+ - Dysphagia
+ - Adults
+ - May be asymptomatic
+ - Esophageal obstruction (i.e., dysphagia)
+- ## Demographics
+
+
+ - Most common symptomatic vascular ring
+ - Typically manifests in neonates
+ - Affects 0.05-0.3% of general population
+- ## Treatment
+
+
+ - Surgical division of smaller or atretic aortic arch and ligamentum arteriosus
+
+ af9c9eb2-551e-4968-a9d6-7738cac6154b
+
+## References
+
+# Selected References
+
+1. [Gikandi A et al: Outcomes of patients undergoing surgery for complete vascular rings. J Am Coll Cardiol. 84(14):1279-92, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39322321%5Bpmid%5D)
+1. [Li S et al: Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification. Cardiovasc Pathol. 39:38-50, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30623879%5Bpmid%5D)
+1. [Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27860551%5Bpmid%5D)
+1. [Newman B et al: Persistent fifth arch anomalies - broadening the spectrum to include a variation of double aortic arch vascular ring. Pediatr Radiol. 46(13):1866-72, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27669708%5Bpmid%5D)
+1. [Ramos-Duran L et al: Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol. 198(5):W466-74, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22528928%5Bpmid%5D)
+1. [Dillman JR et al: Common and uncommon vascular rings and slings: a multi-modality review. Pediatr Radiol. 41(11):1440-54; quiz 1489-90, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21594540%5Bpmid%5D)
+1. [Kanne JP et al: Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 4(5):293-300, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20829147%5Bpmid%5D)
+1. [Kellenberger CJ: Aortic arch malformations. Pediatr Radiol. 40(6):876-84, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20354848%5Bpmid%5D)
+1. [Holmes KW et al: Magnetic resonance imaging of a distorted left subclavian artery course: an important clue to an unusual type of double aortic arch. Pediatr Cardiol. 27(3):316-20, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16565909%5Bpmid%5D)
+1. [Chan MS et al: Angiography and dynamic airway evaluation with MDCT in the diagnosis of double aortic arch associated with tracheomalacia. AJR Am J Roentgenol. 185(5):1248-51, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16247144%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches at thoracic inlet (i.e., 2 carotids and 2 subclavian arteries), the so-called 4-artery sign. This sign has been described in the setting of DAA. Note the right aortic arch (RAA) .*
+
+
+*Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches at thoracic inlet (i.e., 2 carotids and 2 subclavian arteries), the so-called 4-artery sign. This sign has been described in the setting of DAA. Note the right aortic arch (RAA) .*
+
+
+*Composite axial CTA in the same patient shows the larger RAA and smaller left aortic arch . Note the left descending thoracic aorta , which is typically contralateral to the dominant arch.*
+
+
+*Coronal CTA in the same patient shows a cephalad, larger RAA and a more caudal, smaller left aortic arch . Note the mild tracheal impression of the RAA. A larger RAA is the most common variant seen in DAA.*
+
+
+*Sagittal CTA in the same patient shows the distal RAA causing a posterior indentation of the trachea. Also note the ascending aorta (with slab artifact) and the proximal left aortic arch .*
+
+
+*Frontal radiograph in the same patient shows mild concentric narrowing of the midtrachea with more prominent right paratracheal nodular opacity.*
+
+
+*Lateral radiograph in the same patient shows the posterior tracheal indentation from retrotracheal course of the RAA, a classic finding in DAA. This narrowing is often related to the distal portion of the RAA, as it courses posterior to the esophagus to join the RAA.*
+
+
+*Sagittal 3D reformation of chest CTA shows a patent RAA and smaller left aortic arch . There are 4 major symmetrical branches (2 ventral carotids and 2 dorsal subclavian arteries), each set arising form each aortic arch. This is known as the 4-artery sign. The trachea and esophagus (not shown) are completely surrounded by the vascular ring.*
+
+
+*Graphic shows a DAA with a complete vascular ring encircling and compressing the trachea and esophagus.*
+
+
+*Esophagram in a neonate with stridor shows right and smaller left indentations of the esophagus on frontal view due to a DAA. There is posterior indentation in the lateral view related to the RAA.*
+
+
+*Frontal 3D reformation from a chest CT in an asymptomatic patient with a DAA shows higher and larger right vs. left tracheal indentations on the AP reformation. Note posterior indentation related to the RAA in the lateral reformation.*
+
+
+*Lateral chest radiograph in a patient with a DAA shows abnormal posterior tracheal indentation . This can also be seen in the setting of other vascular rings, such as those with diverticulum of Kommerell or pulmonary artery sling.*
+
+
+*Composite axial CTA at contiguous levels shows the RAA and left aortic arch similar in size. Note, however, the lack of the 4-artery sign (i.e., lack of symmetry of the 4 head/neck vessels at the thoracic inlet ). There is a left descending thoracic aorta .*
+
+
+*Frontal chest radiograph in a patient with a DAA with an atretic left arch shows right paratracheal opacity related to the RAA with marked tracheal indentation . The same imaging finding can also be seen in an isolated RAA.*
+
+
+*Composite axial CTA at contiguous levels in the same patient shows symmetric take-off of 4 aortic branches at the thoracic inlet (i.e., 4-artery sign). Note the presence of a large RAA , which is mildly deviating the trachea to the left.*
+
+
+*Composite axial CTA at contiguous levels in the same patient shows an atretic left aortic arch with a posteriorly tethered left subclavian artery . The later is helpful to differentiate from an RAA with mirror image branching in which the take-off of the left subclavian artery tends to be more anterior.*
+
+
+*Sagittal oblique SSD CTA in the same patient shows the posterior tethering of the left subclavian artery and the atretic left aortic arch , resulting in a vascular ring.*
+
+
+### Additional Images
+
+
+*Axial chest CTA in an asymptomatic adult patient with a DAA with areas of partially atretic left aortic arch (cephalad to caudad progression) demonstrates a RAA and a left aortic arch .*
+
+
+*Axial chest CTA in the same patient shows that the RAA and left aortic arch have joined into 1 descending thoracic aorta . The trachea remains slightly narrowed.*
+
+
+*Axial chest CTA more inferiorly shows a common left descending thoracic aorta . The trachea now resumes a normal diameter.*
+
diff --git a/docs_md/articles/ductus-diverticulum_9b1101bc-83a2-445c-aef5-53d633e5bec0.md b/docs_md/articles/ductus-diverticulum_9b1101bc-83a2-445c-aef5-53d633e5bec0.md
new file mode 100644
index 0000000..0658db0
--- /dev/null
+++ b/docs_md/articles/ductus-diverticulum_9b1101bc-83a2-445c-aef5-53d633e5bec0.md
@@ -0,0 +1,416 @@
+---
+title: "Ductus Diverticulum"
+docid: "9b1101bc-83a2-445c-aef5-53d633e5bec0"
+authors:
+ - key: "a354e6da-2757-40e8-b7ff-5e6fb6413ff6"
+ value: "Sachin S. Saboo, MD, FRCR, FSCMR"
+ - key: "770e1d77-2287-436e-910b-48232afc7842"
+ value: "Prabhakar Rajiah, MBBS, MD, FACR, FRCR, FACC, FAHA, FSCCT"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Ductus Diverticulum"
+ slug: "ductus-diverticulum"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "f74b3068-f527-45c0-b021-5088d30db81f"
+imageCount: 19
+lastUpdated: "01/24/25"
+pageDescription: "Ductus Diverticulum"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Ductus Diverticulum"
+pageTitle: "Ductus Diverticulum | STATdx"
+enhancedTitle: "Ductus Diverticulum"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Ductus Diverticulum"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Smooth focal bulge along anteromedial aspect of aortic isthmus at site of obliterated ductus arteriosus
+- ## Imaging
+
+
+ - Chest radiography
+ - Frontal: Opacity in aortopulmonary window
+ - Lateral: Small, bump-like opacity at distal transverse aortic arch
+ - Contrast-enhanced CTA or MRA
+ - Differentiate between typical and atypical appearances
+ - Evaluate for aneurysmal dilatation
+ - Differentiate from traumatic pseudoaneurysm
+ - Ductus diverticulum aneurysm
+ - Saccular dilatation along anterior inferior margin of aortic isthmus
+ - Superior margin of aneurysm extends to left subclavian artery
+ - Differentiate from traumatic pseudoaneurysm
+ - Presence of smooth, uninterrupted margins of diverticulum
+ - No dissection flap
+ - Absence of mediastinal or periaortic hematoma
+- ## Top Differential Diagnoses
+
+
+ - Aortic isthmus (traumatic) pseudoaneurysm
+ - Aortic ulcerated atherosclerotic plaque
+ - Aortic aneurysm
+ - Kommerell diverticulum
+ - Patent ductus arteriosus
+- ## Clinical Issues
+
+
+ - Typically incidental finding
+ - Most patients are asymptomatic
+ - Aneurysmal dilatation of ductus diverticulum necessitates intervention if > 3 cm
+ - Endovascular stent graft or conventional open surgical repair
+
+# TERMINOLOGY
+
+- ## Synonyms
+
+
+ - Ductus bulge; ductus bump
+- ## Definitions
+
+
+ - Smooth focal bulge along anteromedial aspect of aortic isthmus at site of obliterated ductus arteriosus/ligamentum arteriosum
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Well-defined, smooth, broad-based outpouching from anteromedial aspect/lesser curvature of aortic isthmus with obtuse angle with aorta
+ - Mediastinum and aorta are otherwise unremarkable
+ - ### Location
+
+
+ - Along anteromedial aspect of aortic isthmus
+ - ### Size
+
+
+ - Usually small bulge
+ - May increase aortic diameter by average of 4.3 mm
+ - Unusually enlarged ductus referred to as aneurysm
+ - Aneurysmal dilatation of ductus diverticulum > 3 cm needs surgical intervention
+ - ### Morphology
+
+
+ - Smooth bulging of aortic side of ductus arteriosus
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - 3D CT or MR angiography (CTA, MRA)
+ - ### Protocol advice
+
+
+ - Contrast-enhanced CTA
+ - Sagittal oblique thin-slice (≤ 1 mm) reconstructed images
+ - Essential to identify and assess relationship of ductus with pulmonary artery (PA), aortic arch, and subclavian artery (SCA)
+ - Visualize smooth shoulders of ductus diverticulum
+ - Volume-rendering technique (VRT)
+ - To convey 3D anatomic relationships with adjacent vessels and structures than MIP
+ - Cinematic-rendered visualization
+ - 3D visualization methodology with increased surface detail of anatomy and more realistic shadowing effects than can be achieved with VRT
+ - Noncontrast 3D slap MRA or contrast-enhanced MRA
+ - Sagittal oblique and coronal thin reconstructed MIP
+ - Consider sagittal oblique and axial black-blood imaging
+- ## Radiographic Findings
+
+
+ - Frontal chest radiograph
+ - May manifest as opacity in aortopulmonary window
+ - Lateral chest radiograph
+ - Small, bump-like opacity at distal arch/isthmus
+- ## CT Findings
+
+
+ - General
+ - Best visualized on sagittal oblique reconstructed images
+ - May be difficult to identify ductus diverticulum on axial due to partial volumining from oblique orientation
+ - Typical appearance
+ - Broad-based, contrast-filled outpouching at anteromedial aortic isthmus extending blindly inferiorly toward main PA
+ - Best clue: Smooth, uninterrupted margins; gently sloping, symmetric shoulders; obtuse angles with aorta at its superior and inferior margins
+ - Increase in aortic lumen ≤ 1 cm
+ - Smaller vertical height diameter (5.5 mm vs. 11.2 mm) and broader base (14.9 mm vs. 8.8 mm) compared with traumatic pseudoaneurysm
+ - Atypical appearance
+ - Steep and asymmetric sloping
+ - Acute angles at superior margin with loss of gentle superior angle
+ - Ductus may fold back against aorta and result in pseudointimal flap
+ - Ductus diverticulum aneurysm
+ - Saccular dilatation along inferior margin of aortic isthmus opposite origin of left SCA
+ - Superior margin of aneurysm extends to left SCA
+ - Axial CTA images may show typical 3-star sign at aortopulmonary window
+ - Proximal arch, descending aorta, and saccular aneurysm of diverticulum appear as hook-shaped structure
+ - Detection of small pedicle/fibrotic portion of ductus linking aneurysm to PA differentiate ductal aneurysm from aneurysm of aorta
+ - Partial thrombosis of ductus diverticulum aneurysm can be FDG avid on 18F-FDG PET/CT due to inflammation of wall
+ - Differentiate from traumatic pseudoaneurysm
+ - Presence of smooth, uninterrupted margins with smaller vertical height, obtuse angle with aorta in ductus diverticulum
+ - Absence of dissection flap
+ - Absence of mediastinal or periaortic hematoma
+- ## MR Findings
+
+
+ - ### MRA
+
+
+ - MRA and postcontrast GRE images help exclude pseudoaneurysm from atypical ductus diverticulum
+ - Findings similar to those on CTA
+ - Smooth outpouching at anteromedial aspect of aortic isthmus
+ - No dissection flap
+- ## Angiographic Findings
+
+
+ - Contrast-filled, well-defined smooth outpouching arising from anterior inferior margin of aortic isthmus
+ - No dissection flap
+ - Pseudodissection flap may be seen with diverticulum that is folded over
+ - Contrast retention is rarely seen in atypical ductus diverticulum on delayed angiogram views
+ - Typically occurs in traumatic pseudoaneurysm
+ - Aneurysm of ductus diverticulum
+ - Saccular dilatation along anterior inferior margin of aortic isthmus
+ - Superior margin of aneurysm extends to left SCA
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Pseudoaneurysm at Aortic Isthmus (Pseudoductus)
+
+
+ - Due to partial or complete aortic transection
+ - Contrast-filled, irregular outpouching
+ - Varying size/shape; relatively longer vertical diameter
+ - Due to focal disruption of intima and media
+ - Narrow base and acute angles at cranial and caudal ends
+ - Intimal flap in underlying aorta
+ - Mediastinal or periaortic hematoma
+ - May compress aortic lumen
+ - Delayed clearance of contrast on angiography
+- ## Ulcerated Atherosclerotic Plaque at Aortic Isthmus
+
+
+ - Contrast-filled, irregular outpouching
+ - Commonly associated with mural thickening and Ca⁺⁺
+ - Solitary or multifocal
+ - Typically seen in older adult patients
+- [Aortic Aneurysm](/document/aortic-aneurysm/54f93fcd-d960-4770-8d02-1923adb2c01c)
+ - Typically atherosclerotic in etiology, seen in older adults
+ - Not usually localized to region of ductus
+ - Saccular aneurysm involves anterolateral aorta
+ - Absence of small fibrotic pedicle seen with ductal aneurysm
+- ## Kommerell Diverticulum
+
+
+ - Dilatation/aneurysm of aberrant right/ left SCA origin
+ - May be associated with right aortic arch and vascular ring
+- [Patent Ductus Arteriosus](/document/patent-ductus-arteriosus/5ba3261d-bd13-4542-92ec-5db5274e2050)
+ - Beyond 3 months after birth
+ - Left-to-right shunt via funnel, tubular or window-type connection
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - In developing fetus, ductus arteriosus connects PA to descending aorta for right-to-left shunt
+ - Allows most of blood from right ventricle to bypass fetal lungs in utero
+ - Normally closes after birth, functionally within 24 to 48 hours and anatomically in 1 week to 2 months
+ - Ductus diverticulum is embryologic remnant of infundibular part of ductus arteriosus or remnant of right dorsal aortic root
+ - Located at transition from aortic arch to descending aorta called aortic isthmus
+ - Aortic isthmus is slightly constricted part of proximal descending thoracic aorta immediately distal to left SCA at attachment point of ductus arteriosus
+ - Aortic spindle is small, circumferential bulge just below aortic isthmus
+ - ### Associated abnormalities
+
+
+ - Aneurysm of ductus diverticulum
+ - Patent ductus arteriosus
+ - Ductus diverticulum common (21%) in acute type B aortic dissection (TBAD)
+ - Primary entry tears in acute TBAD located at ductus diverticulum orifice
+- ## Staging, Grading, & Classification
+
+
+ - Classification based on appearance
+ - Typical, atypical
+ - Classification of aortic isthmus
+ - Type I: Concave contour of aortic isthmus with parallel walls and uniform diameter; most common type
+ - Type II: Mild straightening or convexity of aortic isthmus without discrete bulge
+ - Type III: Ductus diverticulum: Discrete focal bulge of aortic isthmus least common type (8-26%)
+- ## Microscopic Features
+
+
+ - Remnant of infundibular part of ductus arteriosus
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Asymptomatic
+ - Typically incidental finding
+ - ### Other signs/symptoms
+
+
+ - Aneurysmal dilatation
+ - Embolic stroke, compression of local thoracic structures producing cough, hoarseness, dyspnea, chest pain, neck pain, dysphagia
+ - Rupture may lead to hemodynamic instability
+- ## Demographics
+
+
+ - Age: More common in children than in adults
+ - Dissection patients with ductus diverticulum were relatively younger than TBAD alone
+ - Sex: M = F
+ - Epidemiology
+ - 33% of infants
+ - 9-26% of adults in angiography study
+- ## Natural History & Prognosis
+
+
+ - Diverticulum usually shrinks over time
+ - Small, residual bump at isthmus
+ - Rarely ductus aneurysm formation in
+ - Hypertensive and older adults with atherosclerotic aorta
+ - Behçet disease, Marfan and Ehlers-Danlos syndromes
+ - Following surgical closure of patent ductus arteriosus
+ - Rupture, dissection, thromboembolism, phrenic nerve compression, and infection of aneurysm may occur
+ - Ligamentum arteriosum (fibrous band) develops from obliteration of ductus arteriosus at aortic isthmus and can develop linear calcification
+- ## Treatment
+
+
+ - Usually no treatment required
+ - Aneurysmal dilatation of ductus diverticulum → intervention if > 3 cm, or enlarging or symptomatic
+ - Endovascular stent graft repair
+ - Conventional open surgical repair
+ - Endovascular coil embolization if standard thoracic endovascular aortic repair (TEVAR) method is unsuccessful
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Image Interpretation Pearls
+
+
+ - Best imaging tool: 3D CT or MR angiography (CTA, MRA)
+ - Best visualized on sagittal oblique reformatted CTA/MRA/angiography images
+ - Differentiate from traumatic pseudoaneurysm
+ - Smooth, uninterrupted margins, broad base with aorta, smaller vertical height
+ - Absence of dissection flap
+ - Absence of mediastinal or periaortic hematoma
+
+ a73cc84a-87fa-4d1c-aa19-e7d897f011c2
+
+## References
+
+# Selected References
+
+1. [Chen D et al: Association of ductus diverticulum and acute type B aortic dissection. Acad Radiol. 30(11):2541-7, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36754645%5Bpmid%5D)
+1. [Celik E et al: The aortic ductus diverticulum-innocent bystander or potential source of thromboembolic stroke? J Comput Assist Tomogr. 46(3):392-6, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35575652%5Bpmid%5D)
+1. [Buechner D et al: Successful endovascular coil embolization of large pseudoaneurysm of ductus arteriosus diverticulum. CVIR Endovasc. 2(1):12, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=32026144%5Bpmid%5D)
+1. [Rowe SP et al: MDCT of ductus diverticulum: 3D cinematic rendering to enhance understanding of anatomic configuration and avoid misinterpretation as traumatic aortic injury. Emerg Radiol. 25(2):209-13, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29327106%5Bpmid%5D)
+1. [Thampy R et al: Thrombosed aneurysm of the ductus diverticulum mimicking malignancy on 18F-FDG PET/CT. BMJ Case Rep. 11(1), 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30598473%5Bpmid%5D)
+1. [Nagpal P et al: Advances in imaging and management trends of traumatic aortic injuries. Cardiovasc Intervent Radiol. 40(5):643-54, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28078377%5Bpmid%5D)
+1. [Hyung Ann J et al: Morphologic evaluation of ductus diverticulum using multi - detector computed tomography: comparison with traumatic pseudoaneurysm of the aortic isthmus. Iran J Radiol. 13(4):e38016, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27895881%5Bpmid%5D)
+1. [Modi A et al: Dissection from ductus diverticulum presenting as type A intramural hematoma. Asian Cardiovasc Thorac Ann. 22(1):107, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24585659%5Bpmid%5D)
+1. [Agarwal PP et al: Multidetector CT of thoracic aortic aneurysms. Radiographics. 29(2):537-52, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19325064%5Bpmid%5D)
+1. [Vogler T et al: [Diverticulum of the ductus arteriosus. Cause of traumatic aortic ruptures?.] Chirurg. 78(1):47-51, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17151844%5Bpmid%5D)
+1. [Saito N et al: Successful endovascular repair of an aneurysm of the ductus diverticulum with a branched stent graft: case report and review of literature. J Vasc Surg. 40(6):1228-33, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15622379%5Bpmid%5D)
+1. [Gotway MB et al: Thoracic aorta imaging with multisclice CT. Radiol Clin North Am. 41(3):521-43, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12797604%5Bpmid%5D)
+1. [Sugimoto T et al: Aneurysm of the ductus diverticulum in adults: the diagnostic value of three-dimensional computed tomographic scanning. Jpn J Thorac Cardiovasc Surg. 51(10):524-7, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14621015%5Bpmid%5D)
+1. [Batra P et al: Pitfalls in the diagnosis of thoracic aortic dissection at CT angiography. Radiographics. 20(2):309-20, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10715333%5Bpmid%5D)
+1. [Ferrera PC et al: Ductus diverticulum interpreted as traumatic aortic injury. Am J Emerg Med. 15(4):371-2, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9217528%5Bpmid%5D)
+1. [Fisher RG et al: "Lumps" and "bumps" that mimic acute aortic and brachiocephalic vessel injury. Radiographics. 17(4):825-34, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9225385%5Bpmid%5D)
+1. [Oxorn D et al: The ductus diverticulum: false-positive angiographic diagnosis of traumatic aortic disruption. J Cardiothorac Vasc Anesth. 11(1):86-8, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9058228%5Bpmid%5D)
+1. [Grollman JH: The aortic diverticulum: a remnant of the partially involuted dorsal aortic root. Cardiovasc Intervent Radiol. 12(1):14-7, 1989](http://www.ncbi.nlm.nih.gov/pubmed/?term=2496921%5Bpmid%5D)
+1. [Morse SS et al: Traumatic aortic rupture: false-positive aortographic diagnosis due to atypical ductus diverticulum. AJR Am J Roentgenol. 150(4):793-6, 1988](http://www.ncbi.nlm.nih.gov/pubmed/?term=3258092%5Bpmid%5D)
+1. [Goodman PC et al: Angiographic evaluation of the ductus diverticulum. Cardiovasc Intervent Radiol. 5(1):1-4, 1982](http://www.ncbi.nlm.nih.gov/pubmed/?term=6805955%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Graphic demonstrates normal anatomy of the great vessels and the presence of a ductus diverticulum (DD) , part of the remnant of the embryologic ductus arteriosus that connected the pulmonary arteries and the aorta in utero. The rest of the ductus becomes the ligamentum arteriosum .*
+
+
+*Graphic demonstrates normal anatomy of the great vessels and the presence of a ductus diverticulum (DD) , part of the remnant of the embryologic ductus arteriosus that connected the pulmonary arteries and the aorta in utero. The rest of the ductus becomes the ligamentum arteriosum .*
+
+
+*Axial (left) and sagittal oblique (right) images in a 85-year-old woman show a broad-based DD with rim calcification. This is a common finding in patients of all ages and should not be confused with pathology.*
+
+
+*Axial CECT shows a typical DD arising from the very proximal portion of the descending thoracic aorta (DTA), just distal to the left subclavian artery.*
+
+
+*Coronal reformatted CECT demonstrates a typical DD . The close proximity of the DD to the pulmonary trunk reflects its underlying etiology as the remnant of the infundibular part of the ductus arteriosus that connected the pulmonary artery to the aortic arch in utero.*
+
+
+*CECT though the inferior aspect of the proximal DTA shows a partially thrombosed 4.8-cm ductal aneurysm (DA) with compression of the left pulmonary artery on coronal oblique image.*
+
+
+*Axial CECT (left) shows spontaneous contained rupture of a 6 x 4 cm DA with surrounding hematoma. Sagittal oblique CECT (right) shows the large DA with small calcifications along its edge, which are common. There is no aortic atherosclerotic disease. (Courtesy S. Kligerman, MD.)*
+
+
+*Axial black-blood MR of an asymptomatic patient demonstrates focal outpouching of the anterior wall of aortic isthmus.*
+
+
+*Sagittal black-blood MR in the same patient shows smooth outpouching from the anteromedial wall of aortic isthmus. These findings are classic for a DD. The absence of an intimal flap and the lack of mediastinal or periaortic hematoma essentially exclude the possibility of a traumatic pseudoaneurysm.*
+
+
+*Axial CECT demonstrates focal outpouching from the anterior wall of the aortic isthmus, consistent with a typical DD.*
+
+
+*Axial CECT shows a partially thrombosed DD aneurysm . Although most patients are asymptomatic and require no treatment, the presence of aneurysmal dilatation > 3 cm necessitates endovascular stent graft or open surgical repair.*
+
+
+### Additional Images
+
+
+*Sagittal CTA following endovascular repair of the DD aneurysm shows a thoracic aortic stent graft and exclusion of the DA .*
+
+
+*Sagittal CTA shows a smooth, well-defined wide-based outpouching from the aortic isthmus, consistent with a DD. The DD is located at the transition from the distal aortic arch to the descending aorta.*
+
+
+*Sagittal CTA shows a small bulge with gentle obtuse angles at the aortic wall, which is typical for DD . The proximity of the DD to the main pulmonary artery reflects that it is a remnant of the ductus arteriosus that connected the pulmonary artery to the aortic arch in utero.*
+
+
+*Sagittal CTA demonstrates an atypical DD, which forms acute angles with the aortic wall. However, the smooth, uninterrupted margin with the aortic wall is consistent with this benign diagnosis.*
+
+
+*Axial CECT in the same patient shows the atypical DD . In a setting of trauma, the lack of periaortic or mediastinal hematoma and the absence of an intimal flap aid in differentiating this from aortic pseudoaneurysm.*
+
+
+*Sagittal CTA in the same patient shows the outpouching from the anteromedial wall of aortic isthmus, which is consistent with a thrombosed ductus DA . Although such aneurysms are rare, they can occur in older adult hypertensive patients with an atherosclerotic aorta.*
+
+
+*Candy cane view of the thoracic aorta shows a small DD just distal to aortic isthmus. Note postoperative replacement of ascending aorta for type A aortic dissection.*
+
+
+*Oblique sagittal CTA of the thoracic aorta shows type B aortic dissection with incidental note made of the DD arising from a false lumen.*
+
+
+*VRT of the thoracic aorta shows type B aortic dissection with incidental note made of the DD arising from a false lumen.*
+
diff --git a/docs_md/articles/enlarged-cardiac-silhouette_13d59c6a-0f7f-4389-8d8c-f19ca039e446.md b/docs_md/articles/enlarged-cardiac-silhouette_13d59c6a-0f7f-4389-8d8c-f19ca039e446.md
new file mode 100644
index 0000000..33565c0
--- /dev/null
+++ b/docs_md/articles/enlarged-cardiac-silhouette_13d59c6a-0f7f-4389-8d8c-f19ca039e446.md
@@ -0,0 +1,109 @@
+---
+title: "Enlarged Cardiac Silhouette"
+docid: "13d59c6a-0f7f-4389-8d8c-f19ca039e446"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Enlarged Cardiac Silhouette"
+ slug: "enlarged-cardiac-silhouette"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "c4c4d705-71ba-4631-be39-f4b35c596710"
+imageCount: 5
+lastUpdated: "03/17/22"
+pageDescription: "Enlarged Cardiac Silhouette"
+pageKeywords: "Cardiac, Differential Diagnosis, Enlarged Cardiac Silhouette"
+pageTitle: "Enlarged Cardiac Silhouette | STATdx"
+enhancedTitle: "Enlarged Cardiac Silhouette"
+type: "DDX"
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Enlarged Cardiac Silhouette"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Pericardial space fluid: Globular enlargement
+ - Cardiac chamber enlargement: Characteristic contour abnormality, such as filling of retrosternal clear space in right ventricle enlargement
+ - Pericardial mass: Focal contour irregularity
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Ischemic Cardiomyopathy**
+ - Subendocardial fat or calcium, left ventricle (LV) wall thinning in coronary distribution, dense coronary calcifications
+ - MR shows subendocardial or transmural delayed enhancement in coronary artery distribution
+ - **Valvular Disease**
+ - Valvular calcifications most common
+ - MR cine or phase contrast shows flow jets
+ - **Heart Failure Exacerbation**
+ - Coexistent signs of pulmonary edema
+ - **Pericardial****Effusion**
+ - New globular heart enlargement on radiograph, fluid-density pericardial fluid on CT
+ - Hemopericardium suggested by high-density pericardial fluid or neoplasm history (lung, breast, melanoma)
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Nonischemic Dilated Cardiomyopathy**
+ - Dilated LV, thin wall, LVEF < 40%
+ - Either no delayed enhancement present or enhancement is not subendocardial
+ - **Pericardial Mass**
+ - Pericardial cyst: Circumscribed fluid density at right more than left cardiophrenic angle
+ - Pericardial fat pad: Fat density most commonly at right cardiophrenic angle
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Left Ventricle Aneurysm**
+ - True aneurysm
+ - Post infarct wall thinning, dilatation, and associated thrombus
+ - Most often present along apical anterior or lateral wall
+ - False aneurysm
+ - Ruptured myocardium contained by pericardial adhesions
+ - Most commonly seen at infero-lateral or inferior basal LV wall segments
+ - Neck narrower than internal diameter
+
+
+## Images
+
+
+### Selected Images
+
+
+**Ischemic Cardiomyopathy**
+*Short-axis delayed contrast-enhanced image shows subendocardial enhancement in the septal and anterior wall at the base. The patient had hypokinesis and wall thinning at this location.*
+
+
+**Ischemic Cardiomyopathy**
+*Short-axis delayed contrast-enhanced image shows subendocardial enhancement in the septal and anterior wall at the base. The patient had hypokinesis and wall thinning at this location.*
+
+
+**Valvular Disease**
+*Axial CECT shows an enlarged right atrium in a patient with severe tricuspid regurgitation. Radiograph showed rightward deviation of the right heart border. Regurgitant jet was seen on MR.*
+
+
+### Additional Images
+
+
+**Heart Failure Exacerbation**
+*Frontal radiograph shows a normal cardiac silhouette in a patient with ischemic cardiomyopathy and a 40% ejection fraction. Compare with the next image, which was acquired 1 month later.*
+
+
+**Heart Failure Exacerbation**
+*Frontal radiograph shows marked enlargement of the cardiac silhouette. The patient presented with increasing shortness of breath. Large pericardial effusion was found with presumed etiology of heart failure exacerbation.*
+
+
+**Left Ventricle Aneurysm**
+*Frontal radiograph shows left cardiac border contour deformity . CT showed abdominal fat, which had herniated into the left cardiophrenic space.*
+
diff --git a/docs_md/articles/giant-cell-arteritis_208eca17-81b8-448c-b8be-80e274dccc42.md b/docs_md/articles/giant-cell-arteritis_208eca17-81b8-448c-b8be-80e274dccc42.md
new file mode 100644
index 0000000..2fcc3e8
--- /dev/null
+++ b/docs_md/articles/giant-cell-arteritis_208eca17-81b8-448c-b8be-80e274dccc42.md
@@ -0,0 +1,440 @@
+---
+title: "Giant Cell Arteritis"
+docid: "208eca17-81b8-448c-b8be-80e274dccc42"
+authors:
+ - key: "ee6ece9d-ad74-458c-a8df-11628ae7f879"
+ value: "Arzu Canan, MD"
+ - key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
+ value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
+ - key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
+ value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Giant Cell Arteritis"
+ slug: "giant-cell-arteritis"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "9aabd711-33a6-4598-b5e2-495eff0adf14"
+imageCount: 16
+lastUpdated: "11/14/24"
+pageDescription: "Giant Cell Arteritis"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Giant Cell Arteritis"
+pageTitle: "Giant Cell Arteritis | STATdx"
+enhancedTitle: "Giant Cell Arteritis"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Giant Cell Arteritis"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Chronic, systemic, large or medium-sized, often granulomatous vasculitis
+ - Often involves thoracic aorta and major branches
+ - Often involves temporal artery
+- ## Imaging
+
+
+ - CTA
+ - Concentric aortic thickening (> 2 mm)
+ - Aortic aneurysm; classically ascending aorta
+ - Aortic dissection: Intimomedial flap
+ - MR
+ - Assessment of active inflammation
+ - Delayed enhancement after gadolinium
+ - Ultrasonography
+ - High specificity and sensitivity; operator dependent
+ - Hypoechoic halo temporal &/or axillary arteries
+ - PET
+ - Active inflammation demonstrates ↑ FDG uptake
+- ## Top Differential Diagnoses
+
+
+ - Takayasu arteritis
+ - May be identical to GCA
+ - Extremely rare in patients > 50 years
+ - Atherosclerotic disease
+ - May be difficult to differentiate radiographically, though clinical symptoms often facilitate process
+ - Similar age group
+- ## Clinical Issues
+
+
+ - Headache, visual disturbances, jaw claudication
+ - Polymyalgia rheumatica
+ - Serologic markers
+ - ↑ sedimentation rate
+ - ↑ C-reactive protein
+ - Thrombocytosis
+ - Treatment
+ - Corticosteroids
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Giant cell arteritis (GCA)
+- ## Synonyms
+
+
+ - Temporal arteritis
+ - Cranial GCA (C-GCA) often referred to as temporal arteritis; terminology not longer recommended, as sparing of temporal artery is not uncommon and because disease may involve large vessels
+ - Horton disease
+- ## Definitions
+
+
+ - Granulomatous autoimmune vasculitis affecting larger arteries and aorta
+ - C-GCA: Often involves temporal artery and other head/neck vessels, but may also involve aorta and major branches
+ - Large-vessel GCA (LV-GCA): Often involves thoracic aorta and major branches
+ - Frequently associated with polymyalgia rheumatica (PMR)
+ - Aching and morning stiffness in shoulders, hip girdle, and neck
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Location
+
+
+ - Temporal artery
+ - Aorta and aortic branches
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - Typically, GCA involving aorta is not as apparent or dense as intramural hematoma; however, there can be hyperdensity if associated with hemorrhage or calcification
+ - Transmural calcification is often similar to calcified atherosclerotic plaques (common)
+ - ### CTA
+
+
+ - Concentric aortic thickening (> 2 mm)
+ - Aortic stenosis
+ - Aortic aneurysm; classically ascending aorta
+ - Aortic dissection: Intimomedial flap
+ - Limited role in C-GCA
+- ## MR Findings
+
+
+ - Equally accurate as CT for morphologic assessment on several sequences (e.g., T1WI, T2WI, HASTE, SSFP, etc.)
+ - Contrast-enhanced MRA is more accurate to assess areas of stenosis and aneurysm
+ - Assessment of active inflammation
+ - Contrast-enhanced sequences: Delayed enhancement (i.e., ↑ signal) of vessel wall after gadolinium
+ - Fat-saturated STIR sequence: High signal of thickened vessel wall
+ - Cranial (temporal artery) involvement
+ - High sensitivity and specificity
+ - Mural thickening (> 0.5 mm)
+ - Mural high T2 signal and contrast enhancement
+- ## Ultrasonographic Findings
+
+
+ - ### Grayscale ultrasound
+
+
+ - C-GCA
+ - High specificity and sensitivity; operator dependent
+ - Hypoechoic halo (i.e., **halo sign**) in temporal &/or axillary arteries
+ - **Compression sign**: Persistence of halo during compression of vessel lumen by ultrasound probe
+ - ### Color Doppler
+
+
+ - Always in conjunction with grayscale ultrasound
+ - Helpful to localize temporal artery
+- ## Angiographic Findings
+
+
+ - Stenosis (often long, regular, and smooth-walled)
+ - Occlusion
+ - Aneurysm
+ - Limited in diagnosis of early vasculitis
+- ## Nuclear Medicine Findings
+
+
+ - ### PET
+
+
+ - LV-GCA: Active inflammation demonstrates ↑ FDG uptake
+ - Subclinical inflammation of large vessels in 80% with GCA and ~ 30% PMR
+ - Response to treatment correlates with ↓ FDG uptake
+ - Limited role in C-GCA, not recommended
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - MR
+ - STIR: Thickening and high signal of aortic wall
+ - Contrast-enhanced MR: Thickening and enhancement of aortic wall
+ - MRA is helpful to detect areas of stenosis and aneurysm
+ - ### Protocol advice
+
+
+ - Consider concomitant NECT to differentiate from intramural hematoma
+ - Caveat: GCA can occasionally be hyperdense
+ - PET
+ - Recognized role in patient with fever &/or inflammation of unknown origin
+ - Unclear role in follow-up, especially asymptomatic patients without elevated inflammatory markers
+
+# DIFFERENTIAL DIAGNOSIS
+
+- [Takayasu Arteritis](/document/takayasu-arteritis/3b589c7b-d975-4f2c-a5b1-ff83dd856ee7)
+ - May have similar imaging appearance to GCA
+ - Rare in patients > 50 years old
+- [Other Systemic Vasculitides](/document/polyarteritis-nodosa/5c1ed46f-9132-4903-830d-1907a0774c7d)
+ - e.g., polyarteritis nodosa, syphilitic aortitis
+ - Occurs most often in small and medium-sized arteries
+ - Biopsy and pattern of distribution often help differentiation
+- ## Fibromuscular Dysplasia
+
+
+ - Most often affects renal arteries
+ - Can also involve carotid arteries
+ - Results in stenoses; occasional spontaneous dissection
+- [Atherosclerotic Disease](/document/atherosclerosis/41278e3c-2240-4122-b555-8776d0918082)
+ - May be difficult to differentiate radiographically, though clinical symptoms often facilitate process
+ - Similar age group
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Unknown
+ - Most accepted hypothesis: Antigen-driven disease mediated by T cells and macrophages that reach aortic wall via vasa vasorum
+- ## Staging, Grading, & Classification
+
+
+ - Temporal artery biopsy remains diagnostic gold standard for C-GCA
+ - Predictors of positive temporal artery biopsy
+ - Jaw claudication
+ - Neck pain
+ - C-reactive protein > 2.45 mg/dL
+ - Sedimentation rate > 47 mm/h
+ - Thrombocytosis
+ - Pallid optic disc edema
+ - Temporal artery abnormalities
+ - Temporal artery biopsy can be negative (10-15%)
+- ## Gross Pathologic & Surgical Features
+
+
+ - Involvement of aorta (65.0%)
+ - Involvement of main aortic tributaries (57.5%)
+ - Brachiocephalic trunk (47.5%)
+ - Subclavian arteries (42.5%)
+ - Carotid arteries (35.0%)
+ - Femoral arteries (30.0%)
+ - Splanchnic arteries (22.5%)
+ - Axillary arteries (17.5%)
+ - Iliac arteries (15.0%)
+ - Renal arteries (7.5%)
+- ## Microscopic Features
+
+
+ - Focal chronic inflammatory cell infiltrates
+ - Granulomas in vessel wall formed by CD4(+) T cells and macrophages
+ - Focal areas of intimal hyperplasia
+ - Proliferation of smooth muscle cells, which leads to narrowing of arterial lumen and eventually ischemia
+ - Focal areas of fragmentation of inner elastic lamina
+ - Focal concentric scars around inner elastic lamina
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Headache
+ - Visual disturbances
+ - Jaw claudication
+ - ### Other signs/symptoms
+
+
+ - PMR
+ - Present in 50% of patients at diagnosis of GCA
+ - 20% of PMR will develop GCA
+ - Clinical manifestations
+ - Morning stiffness
+ - Pain (shoulder > hip or neck)
+ - Synovitis and bursitis
+ - Swelling and tenosynovitis
+ - ↓ range of motion
+ - Muscle tenderness
+ - Subjective weakness
+ - Systemic signs and symptoms (e.g., malaise, fatigue, depression, anorexia, weight loss, fever)
+ - ### Clinical profile
+
+
+ - Clinical phenotypes
+ - C-GCA (temporal arteritis with headache and visual disturbance)
+ - LV-GCA (arm/limb claudication, chest pain)
+ - PMR
+ - Phenotypes can overlap
+ - Serologic markers
+ - ↑ erythrocyte sedimentation rate
+ - ↑ C-reactive protein
+ - Thrombocytosis
+ - Association with HLA-DRB1*04
+ - LV-GCA linked to other systematic diseases, such as Behçet disease or hyper-IgG4 syndrome
+ - Factors for aneurysm formation
+ - Aortic insufficiency
+ - Murmur at time of diagnosis
+ - Hyperlipemia
+ - ↑ eritrosedimentation in combination with polymyalgia symptoms
+ - ↑ levels of IL-2
+- ## Demographics
+
+
+ - ### Age
+
+
+ - Patients > 50 years old
+ - Incidence ↑ steadily with age
+ - ### Sex
+
+
+ - Women > men
+ - ### Ethnicity
+
+
+ - More common in people of Northern European and Scandinavian descent
+ - ### Epidemiology
+
+
+ - Prevalence in USA: 1 in 160,000
+ - Lifetime risk of developing GCA in USA: 1% in women and 0.5% in men
+- ## Natural History & Prognosis
+
+
+ - Prognosis for visual recovery is poor
+ - ↑ risk aortic aneurysm formation and dissection: 17-fold and 2.5x higher risk of thoracic and abdominal aortic aneurysms
+ - ↓ survival rate
+ - Involvement of coronary arteries may result in myocardial infarction or congestive heart failure
+ - Bowel necrosis (uncommon)
+ - 15-30% of PMR cases eventually develop GCA
+- ## Treatment
+
+
+ - GCA and PMR: Corticosteroids
+ - Aspirin
+ - Other (2nd-line therapy)
+ - Methotrexate
+ - Azathioprine
+ - Tocilizumab (IL-6 receptor alpha inhibitor)
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - Annual surveillance to assess for aneurysm and dissection
+ - Alternatives for follow-up
+ - Chest radiograph + echocardiogram + abdominal Doppler ultrasound
+ - Contrast-enhanced CT of chest and abdomen
+
+ a728420c-dc69-475b-9a5a-54a5aee00e78
+
+## References
+
+# Selected References
+
+1. [Pepper K: Giant cell arteritis. Postgrad Med. 135(sup1):22-32, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37021621%5Bpmid%5D)
+1. [Braun J et al: The role of 18F-FDG positron emission tomography for the diagnosis of vasculitides. Clin Exp Rheumatol. 36 Suppl 114(5):108-14, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30296989%5Bpmid%5D)
+1. [Dejaco C et al: The spectrum of giant cell arteritis and polymyalgia rheumatica: revisiting the concept of the disease. Rheumatology (Oxford). 56(4):506-15, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27481272%5Bpmid%5D)
+1. [Gomułka K et al: Horton's disease: still an important medical problem in elderly patients: a review and case report. Postepy Dermatol Alergol. 34(5):510-3, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29507571%5Bpmid%5D)
+1. [Buttgereit F et al: Polymyalgia rheumatica and giant cell arteritis: a systematic review. JAMA. 315(22):2442-58, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27299619%5Bpmid%5D)
+1. [Aschwanden M et al: The ultrasound compression sign to diagnose temporal giant cell arteritis shows an excellent interobserver agreement. Clin Exp Rheumatol. 33(2 Suppl 89):S-113-5, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26016760%5Bpmid%5D)
+1. [Khan A et al: Imaging in giant cell arteritis. Curr Rheumatol Rep. 17(8):527, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26113013%5Bpmid%5D)
+1. [Hartlage GR et al: Multimodality imaging of aortitis. JACC Cardiovasc Imaging. 7(6):605-19, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24925329%5Bpmid%5D)
+1. [Schmidt WA: Ultrasound in vasculitis. Clin Exp Rheumatol. 32(1 Suppl 80):S71-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24529335%5Bpmid%5D)
+1. [Jennette JC et al: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 65(1):1-11, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23045170%5Bpmid%5D)
+1. [Blockmans D: Diagnosis and extension of giant cell arteritis. Contribution of imaging techniques. Presse Med. 41(10):948-54, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22795837%5Bpmid%5D)
+1. [Castañer E et al: Imaging findings in pulmonary vasculitis. Semin Ultrasound CT MR. 33(6):567-79, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23168065%5Bpmid%5D)
+1. [Bossert M et al: Aortic involvement in giant cell arteritis: current data. Joint Bone Spine. 78(3):246-51, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21030278%5Bpmid%5D)
+1. [Falardeau J: Giant cell arteritis. Neurol Clin. 28(3):581-91, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20637990%5Bpmid%5D)
+1. [Bley TA et al: Diagnostic value of high-resolution MR imaging in giant cell arteritis. AJNR Am J Neuroradiol. 28(9):1722-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17885247%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial CTA of the chest in a patient with giant cell arteritis (GCA) shows soft tissue density material surrounding the great vessels. (Courtesy C. S. Restrepo, MD.)*
+
+
+*Axial CTA of the chest in a patient with giant cell arteritis (GCA) shows soft tissue density material surrounding the great vessels. (Courtesy C. S. Restrepo, MD.)*
+
+
+*Axial chest CTA in the same patient shows concentric thickening of the thoracic aorta, which is a common finding in patients with GCA but is indistinguishable from Takayasu arteritis. GCA is more common in patients > 50 years old. Concomitant NECT is recommended to help differentiate from intramural hematoma. (Courtesy C. S. Restrepo, MD.)*
+
+
+*Coronal FDG PET/CT in the same patient shows marked uptake of FDG along the ascending aortic wall . FDG PET has excellent sensitivity and specificity for the diagnosis of GCA and may be used when clinical or serological discrepancies arise during or after treatment of this condition.*
+
+
+*Coronal FDG PET/CT in a patient with GCA shows diffuse uptake along the ascending aortic wall as well as along the subclavian and axillary arteries bilaterally .*
+
+
+*Axial CTA in a patient with GCA shows diffuse arterial wall thickening and stranding of the periaortic fat. Note the reactive left pleural effusion .*
+
+
+*Axial double inversion recovery FS MR in the same patient at different levels shows diffuse high signal of the aortic wall as well as head and neck vessels . MR is the preferred method to assess for active inflammation also seen in the form of vessel parietal enhancement after intravenous gadolinium.*
+
+
+*Sagittal reformat CECT in a patient with GCA before and after contrast shows focal parietal thickening along the posterior descending thoracic aorta, only evident on CECT . Typically, vasculitis is not hyperdense on NECT as opposed to intramural hematoma.*
+
+
+*3D GRE MR (unenhanced and post contrast) at the same level shows progressive enhancement of the aortic wall after administration of intravenous contrast .*
+
+
+*Axial CTA in a patient with unsuspected GCA who underwent reconstruction of the ascending aorta due to aneurysm is shown. Note the aneurysmal ascending and descending aorta .*
+
+
+*Sagittal CECT MIP in the same patient shows diffuse aneurysmal thoracic aorta . Note also the aneurysmal right brachiocephalic trunk . Aneurysm is a very common complication of undiagnosed and untreated GCA only evident after resection.*
+
+
+### Additional Images
+
+
+*Axial CTA in a young patient shows mural thickening of the supraaortic great vessels and stranding of the adjacent perivascular fat due to a vasculitis. Note the stenosis of the left common carotid artery .*
+
+
+*Axial GRE MR following gadolinium administration shows marked circumferential mural thickening and enhancement of the descending thoracic aorta , consistent with active arteritis.*
+
+
+*Axial GRE MR following gadolinium administration in the same patient confirms the presence of mural thickening and enhancement of the supraaortic arteries , consistent with active GCA.*
+
+
+*Coronal contrast-enhanced MRA MIP in the same patient confirms multiple stenoses of the proximal pulmonary arteries without intraluminal thrombus. These are nonspecific features that are consistent with a pulmonary vasculitis, including GCA.*
+
+
+*Axial CTA shows circumferential soft tissue thickening of the aortic arch in a patient with GCA. This represents an inflammatory reaction resulting in aortic mural thickening .*
+
+
+*Axial CTA shows irregular mural thickening of the descending thoracic aorta and pulmonary arteries . Mural thickening represents a common sequela of inflammatory arteritis.*
+
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+++ b/docs_md/articles/interrupted-hypoplastic-aorta_100ba59a-24e8-47b4-b2b2-bbfdc0a21e92.md
@@ -0,0 +1,390 @@
+---
+title: "Interrupted/Hypoplastic Aorta"
+docid: "100ba59a-24e8-47b4-b2b2-bbfdc0a21e92"
+authors:
+ - key: "770e1d77-2287-436e-910b-48232afc7842"
+ value: "Prabhakar Rajiah, MBBS, MD, FACR, FRCR, FACC, FAHA, FSCCT"
+ - key: "e915766e-8102-46e4-a33e-c83f8ae12f29"
+ value: "Harold Goerne, MD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Interrupted/Hypoplastic Aorta"
+ slug: "interruptedhypoplastic-aorta"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "00b8d951-9dbc-40d4-9499-c8705f77762e"
+imageCount: 15
+lastUpdated: "01/28/25"
+pageDescription: "Interrupted/Hypoplastic Aorta"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Interrupted/Hypoplastic Aorta"
+pageTitle: "Interrupted/Hypoplastic Aorta | STATdx"
+enhancedTitle: "Interrupted/Hypoplastic Aorta"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Interrupted/Hypoplastic Aorta"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Interrupted aortic arch
+ - Complete luminal and anatomic wall discontinuity between ascending aorta and descending aorta
+ - Hypoplastic aortic arch
+ - Tubular narrowing without luminal discontinuity
+- ## Imaging
+
+
+ - CTA
+ - Best noninvasive modality to assess aortic arch and supraaortic vessels pattern
+ - US
+ - 1st-line modality to assess aortic arch abnormalities
+ - Best diagnostic clue
+ - Interrupted aortic arch
+ - Blind ends in distal ascending aorta and proximal descending aorta with luminal discontinuity in between
+ - Hypoplastic aortic arch
+ - Tubular narrowing of aortic arch; proximal aortic arch < 60% &/or distal aortic arch < 50% of diameter of ascending aorta
+- ## Top Differential Diagnoses
+
+
+ - Coarctation of aorta
+- ## Pathology
+
+
+ - Type A (13%)
+ - Interruption distal to left subclavian artery
+ - Type B (84%)
+ - Interruption between left common carotid artery and left subclavian artery
+ - Type C (3%)
+ - Interruption between brachiocephalic trunk and left common carotid artery
+- ## Clinical Issues
+
+
+ - Rare condition
+ - 1% of congenital heart disease
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Interrupted aortic arch (IAA)
+ - Hypoplastic aortic arch (HAA)
+- ## Synonyms
+
+
+ - Atresia of aortic arch
+- ## Definitions
+
+
+ - IAA
+ - Complete luminal discontinuity between ascending aorta and descending aorta
+ - Not true interruption, as there is fibrotic continuity between blind ends
+ - HAA
+ - Tubular narrowing of aortic arch without luminal discontinuity
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - IAA
+ - Blind-ending distal ascending aorta and proximal descending aorta with luminal discontinuity in between
+ - HAA
+ - Tubular narrowing of aortic arch
+ - Proximal arch < 60% &/or distal aortic arch < 50% of diameter of ascending aorta
+ - Transverse aortic arch z-score usually < -3
+ - ### Location
+
+
+ - Aortic arch: Proximal or distal
+ - ### Size
+
+
+ - Variable
+ - ### Morphology
+
+
+ - IAA: Complete anatomic discontinuity between ascending aorta and descending aorta
+ - HAA: Tubular narrowing of aortic arch
+- ## CT Findings
+
+
+ - ### CTA
+
+
+ - Interruption: Blind ending ascending and descending aorta without luminal opacification of arch
+ - Type, site, and length of interruption
+ - Type A: Distal to left subclavian artery
+ - Type B: Between left common carotid and subclavian arteries
+ - Type C: Between right brachiocephalic and left common carotid arteries
+ - Hypoplasia: Small caliber of aortic arch
+ - Best modality for measuring vessel diameters
+ - Best noninvasive modality to assess aortic arch and supraaortic vessels pattern
+ - New-generation scanners provide faster acquisition without sedation or ECG gating
+ - High-resolution 3D reconstructions provides roadmap for surgery
+- ## MR Findings
+
+
+ - ### MRA
+
+
+ - High-resolution images of aorta without radiation; can be performed ± contrast
+ - Types of interrupted arch
+ - Shows diameters of each aortic segment and length of HAA
+ - Hypoplasia: External diameter of proximal arch, distal arch, or isthmus measuring < 60%, < 50%, or < 40% of that of ascending aorta
+ - Transverse aortic arch z-score usually < -3
+ - This assumes that ascending aorta diameter is normal
+ - Dynamic MRA shows multiple vascular phases, providing information about aorta, pulmonary arteries and veins, and systemic venous return pattern with single gadolinium injection
+ - Associated anomalies can be evaluated
+ - ### MR cine
+
+
+ - Biventricular function assessment, including ejection fraction, end-diastolic, and end-systolic volumes
+ - Wall motion abnormalities
+ - Evaluation of additional intracardiac abnormalities (such as septal defects)
+- ## Ultrasonographic Findings
+
+
+ - 1st modality to assess aortic arch abnormalities in children
+ - Define true interruption or HAA and type of IAA by looking at pattern of supraaortic trunks
+ - Associated patent ductus arteriosus (PDA)
+ - Size, flow, aortic and pulmonary ostial diameters
+ - Associated cardiac abnormalities: Atrial septal defect (ASD), ventricular septal defect (VSD), left ventricular outflow tract (LVOT) obstruction, aorticopulmonary window defect
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - CTA or MRA
+ - ### Protocol advice
+
+
+ - Newborn and infants: Contrast injection based on body weight
+ - Bolus tracking position and HU threshold are variable and depend on scanner speed to start acquisition
+ - Newborn and infants: Acquisition can be started immediately after contrast injection
+
+# DIFFERENTIAL DIAGNOSIS
+
+- [Coarctation of Aorta](/document/coarctation-of-aorta/c0b23d8c-05e3-4373-b5d9-2de1590414a7)
+ - Focal narrowing at aortic isthmus
+ - Same location as type A interruption
+ - Usually short segment of luminal narrowing
+ - No complete loss of continuity, like interruption
+ - Occasionally, extremely tight stenosis may be seen
+ - More pronounced poststenotic dilation
+ - In interruption, arch is smaller caliber, and branch vessels are straighter than normal
+ - With advanced cases, distinguishing features may disappear
+- ## Focal Atresia of Aortic Arch
+
+
+ - Most common at aortic isthmus, similar to type A interruption
+ - Lumen is interrupted, but aortic wall is present
+ - Fibrous strand between ascending and descending aorta
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Type A
+ - Abnormal regression of left 4th aortic arch late in development after left subclavian artery is in position
+ - Reduced blood flow through 4th aortic arch during embryologic phase
+ - Insufficient development of aortic arch with spectrum from coarctation to atresia to IAA
+ - Conal septum not malaligned or deviated; no subaortic stenosis
+ - Type B
+ - High association with chromosome 22q11.2 microdeletion
+ - Abnormal regression of left 4th arch, early in development, before cephalad migration of left subclavian artery
+ - Malalignment of infundibular septum with muscular septum → LVOT narrowing → decreased growth, hypoplasia, and interruption of arch due to absolute decrease in cardiac output
+ - Type C
+ - Abnormal regression of ventral portion of left 3rd and 4th arches
+ - ### Genetics
+
+
+ - 50% of patients with IAA have chromosome 22q11.2 deletion
+ - 42% of patients with DiGeorge syndrome have IAA
+ - ### Associated abnormalities
+
+
+ - IAA
+ - PDA is essential for life in all patients (seen in 97% of cases)
+ - VSD in 90% of IAA
+ - Other congenital heart abnormalities are present in 98%
+ - Subaortic stenosis
+ - Bicuspid aortic valve
+ - Truncus arteriosus
+ - Aortopulmonary window
+ - Transposition of great arteries
+ - Double-outlet right ventricle
+ - Functional single ventricle
+ - Persistent 5th arch
+ - Anomalous origin of subclavian artery
+ - HAA
+ - ASD
+ - VSD
+ - PDA
+- ## Staging, Grading, & Classification
+
+
+ - Type A (13%)
+ - Interruption distal to left subclavian artery
+ - Type B (84%)
+ - Interruption between left common carotid artery and left subclavian artery
+ - Type C (3%)
+ - Interruption between right brachiocephalic trunk and left common carotid artery
+ - In any of these types, 3 subtypes may be seen depending on origin of right subclavian artery
+ - Subtype 1: Normal subclavian artery origin
+ - Subtype 2: Aberrant right subclavian artery distal to origin of left subclavian artery
+ - Subtype 3: Isolated right subclavian artery originating from right ductus arteriosus
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Differential cyanosis (ductal right-to-left shunt)
+ - Type A
+ - Normal saturation in both arms and head, desaturated legs
+ - Type B
+ - Normal saturation in right arm and head, desaturated left arm and legs
+ - Type C
+ - Normal saturation in right arm and right carotid artery, desaturated left carotid artery, left arm and legs
+ - ### Other signs/symptoms
+
+
+ - When ductus arteriosus begins to close, neonate develops signs of hypoperfusion and cardiogenic shock
+ - Death usually occurs 4-10 days after closure of ductus arteriosus
+ - By 1 month, 76% of untreated infants are dead; by 1 year, > 90% are dead
+- ## Demographics
+
+
+ - ### Age
+
+
+ - Neonates
+ - ### Sex
+
+
+ - Male patients: 59%
+ - Female patients: 41%
+ - ### Epidemiology
+
+
+ - Rare condition
+ - 1% of congenital heart disease
+ - 2/100,000 live births
+- ## Natural History & Prognosis
+
+
+ - When untreated and ductus arteriosus closes, distal hypoperfusion leads to renal failure, lactic acidosis, and eventually death in few days
+- ## Treatment
+
+
+ - Surgical correction is only treatment; goal is to establish continuity in aortic arch
+ - Prostaglandin E₁ is given to maintain patency of ductus arteriosus until neonate is stable for surgical correction
+
+ 4e578d09-68e6-4af7-8a56-23527ab96783
+
+## References
+
+# Selected References
+
+1. [Evans WN et al: Prenatal diagnosis of hypoplastic aortic arch without intracardiac malformations: the nevada experience. J Card Surg. 37(11):3705-10, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36047366%5Bpmid%5D)
+1. [LaPar DJ et al: Surgical considerations in interrupted aortic arch. Semin Cardiothorac Vasc Anesth. 22(3):278-84, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29774793%5Bpmid%5D)
+1. [Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27860551%5Bpmid%5D)
+1. [Goudar SP et al: Echocardiography of coarctation of the aorta, aortic arch hypoplasia, and arch interruption: strategies for evaluation of the aortic arch. Cardiol Young. 26(8):1553-62, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=28148317%5Bpmid%5D)
+1. [Roubertie F et al: Aortopulmonary window and the interrupted aortic arch: midterm results with use of the single-patch technique. Ann Thorac Surg. 99(1):186-91, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25440264%5Bpmid%5D)
+1. [Ramos-Duran L et al: Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol. 198(5):W466-74, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22528928%5Bpmid%5D)
+1. [Hellinger JC et al: Congenital thoracic vascular anomalies: evaluation with state-of-the-art MR imaging and MDCT. Radiol Clin North Am. 49(5):969-96, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21889017%5Bpmid%5D)
+1. [Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20631369%5Bpmid%5D)
+1. [Kimura-Hayama ET et al: Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 30(1):79-98, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20083587%5Bpmid%5D)
+1. [Dillman JR et al: Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol. 190(6):1467-74, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18492893%5Bpmid%5D)
+1. [Yang DH et al: Multislice CT angiography of interrupted aortic arch. Pediatr Radiol. 38(1):89-100, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=17965856%5Bpmid%5D)
+1. [Loffredo CA et al: Interrupted aortic arch: an epidemiologic study. Teratology. 61(5):368-75, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10777832%5Bpmid%5D)
+1. [Kaulitz R et al: Echocardiographic assessment of interrupted aortic arch. Cardiol Young. 9(6):562-71, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10593265%5Bpmid%5D)
+1. [Reardon MJ et al: Interrupted aortic arch: brief review and summary of an eighteen-year experience. Tex Heart Inst J. 11(3):250-9, 1984](http://www.ncbi.nlm.nih.gov/pubmed/?term=15227058%5Bpmid%5D)
+1. [CELORIA GC et al: Congenital absence of the aortic arch. Am Heart J. 58:407-13, 1959](http://www.ncbi.nlm.nih.gov/pubmed/?term=13808756%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Posterior oblique CTA cinematic rendering shows type B interrupted aortic arch with an aberrant right subclavian artery . Also note the collateral vessel with right carotid artery . Left carotid artery and left subclavian artery (LSA) are also shown.*
+
+
+*Posterior oblique CTA cinematic rendering shows type B interrupted aortic arch with an aberrant right subclavian artery . Also note the collateral vessel with right carotid artery . Left carotid artery and left subclavian artery (LSA) are also shown.*
+
+
+*Anterosuperior oblique CTA cinematic rendering shows type B interrupted aortic arch (IAA) with aberrant right subclavian artery . Patent ductus arteriosus (PDA) provides blood flow to both subclavian arteries and the distal descending aorta (DA).*
+
+
+*Sagittal oblique MIP CTA shows a hypoplastic aortic arch and PDA .*
+
+
+*Sagittal oblique CTA cinematic rendering in the same patient shows a hypoplastic aortic arch and PDA . CT is the best noninvasive imaging modality to assess aortic arch and supraaortic vessel patterns as well as to measure vessel diameters.*
+
+
+*Anterosuperior oblique CTA cinematic rendering shows type B IAA. The brachiocephalic trunk (BCT) and left carotid artery originate from the proximal aorta. The LSA originates from the ductal arch.*
+
+
+*Sagittal oblique MIP CTA shows type B IAA . The BCT and left common carotid artery (LCC) originate from the proximal aorta. The LSA originates from the ductal arch.*
+
+
+*CTA cinematic rendering demonstrates type B IAA. The LSA originates from the DA . The BCT and LCC originate from the proximal ascending aorta (AA). The PDA provides blood flow to both the LSA and DA.*
+
+
+*Sagittal oblique CTA cinematic rendering shows a hypoplastic distal aortic arch involving the isthmus and proximal DA .*
+
+
+*Neonate with type A IAA shows AA terminating as the BCT , LCC , and LSA (left). Notice the AP window defect between the AA and main pulmonary artery (MPA) . A PDA supplies the DA . PA branches are visible.*
+
+
+*Coronal (left) and sagittal (right) images in a neonate with type B IAA show the AA terminating as the BCT and LCC . The LSA and DA are supplied by a PDA . PA branches arise from the MPA. (Courtesy S. Kligerman, MD.)*
+
+
+### Additional Images
+
+
+*CTA cinematic rendering demonstrates a hypoplastic aortic arch with severe coarctation . Note the decreased diameter of the aortic arch due to hypoplasia.*
+
+
+*Sagittal oblique MIP CTA in the same patient demonstrates decreased diameter of the aortic arch due to a hypoplastic aortic arch and associated severe aortic coarctation .*
+
+
+*Coronal (left) and sagittal (right) oblique images in a 1-day-old with type B IAA show a hypoplastic ascending aorta terminating as the right common carotid and left common carotid arteries. The PDA supplies the descending thoracic aorta (DTA) . A portion of the LSA is seen from the DTA.*
+
+
+*Coronal image in the same patient shows that an aberrant right subclavian artery arises from the DTA distal to the LSA , making this a type B IAA, subtype 2.*
+
+
+*3D image in a neonate with type B IAA shows the ascending aorta terminating as the right BCT and LCC . The PDA supplies the descending thoracic aorta and LSA . (Courtesy S. Kligerman, MD.)*
+
diff --git a/docs_md/articles/intimointimal-intussusception_a7004bbb-3699-4ed4-af21-78f51a3685d9.md b/docs_md/articles/intimointimal-intussusception_a7004bbb-3699-4ed4-af21-78f51a3685d9.md
new file mode 100644
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--- /dev/null
+++ b/docs_md/articles/intimointimal-intussusception_a7004bbb-3699-4ed4-af21-78f51a3685d9.md
@@ -0,0 +1,220 @@
+---
+title: "Intimointimal Intussusception"
+docid: "a7004bbb-3699-4ed4-af21-78f51a3685d9"
+authors:
+ - key: "b00d2bdb-66e1-41ed-90b4-c52904f4d598"
+ value: "Seth Kligerman, MD, MS"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Intimointimal Intussusception"
+ slug: "intimointimal-intussusception"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "178f7921-c162-43ee-b7ae-313021635390"
+imageCount: 4
+lastUpdated: "01/28/25"
+pageDescription: "Intimointimal Intussusception"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Intimointimal Intussusception"
+pageTitle: "Intimointimal Intussusception | STATdx"
+enhancedTitle: "Intimointimal Intussusception"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Intimointimal Intussusception"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Complete circumferential intimal tear in type A > type B aortic dissection
+ - Delaminated intima then rolls upon itself
+- ## Imaging
+
+
+ - Best visualized with ECG-gated CTA
+ - Circumferential intimal tear → unidirectional or bidirectional intimointimal intussusception (IIIS)
+ - Anterograde (43%): Intima superior circumferential intimal tear delaminates upward
+ - Intimal often folded upon itself in aortic arch
+ - Retrograde (56%): Intima inferior to circumferential tear delaminates inferiorly
+ - Intimal may prolapse into LV during diastole
+ - Bidirectional (1%): IIIS both anterograde and retrograde
+ - Between delaminated intima that has intussuscepted superiorly &/or inferiorly, portion of aorta appears "normal" but actually lacks intima
+ - Missing flap or naked aorta sign
+- ## Clinical Issues
+
+
+ - Diagnosis not prospectively made before surgical intervention in 49% of anterograde IIIS and 16% of retrograde IIIS
+ - Anterograde
+ - Chest pain
+ - Neurologic symptoms (62%)
+ - Asymmetric blood pressure (57%)
+ - Retrograde
+ - Myocardial infarction (26.5%)
+ - Severe aortic regurgitation
+ - Mean age: 54 years
+ - M > F
+ - Overall mortality: 24%
+ - Treatment is surgical repair
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Intimointimal intussusception (IIIS)
+- ## Definitions
+
+
+ - Complete circumferential intimal tear in type A aortic dissection; delaminated intima then rolls upon itself like tube sock; given this is circumferential tear, intima can intussuscept anterograde (upward) into aortic arch (AA)/descending thoracic aorta (DTA) &/or retrograde (downward) into aortic root and left ventricle (LV)
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Type A dissection where layers of intimal are folded in on itself within ascending &/or DTA
+ - ### Location
+
+
+ - Ascending aorta or AA (98%)
+ - DTA (2%)
+- ## Radiographic Findings
+
+
+ - No specific radiographic findings are visible other than findings associated with type A dissection
+- ## CT Findings
+
+
+ - Best visualized with ECG-gated CTA
+ - Findings of type A dissection in most cases
+ - Circumferential intimal tear leads to either unidirectional or bidirectional IIIS
+ - Anterograde (43%): Intima superior to level of circumferential intimal tear delaminates upward into AA and DTA
+ - Intimal is often seen folded upon itself in AA or DTA
+ - Intima may obstruct arch vessels leading to neurologic sequela
+ - Retrograde (56%): Intima inferior to level of circumferential tear delaminates inferiorly into aortic root and may prolapse into LV during diastole
+ - Bidirectional (1%): IIIS both anterograde and retrograde
+ - Between delaminated intima that has intussuscepted superiorly &/or inferiorly, there is portion of aorta that appears normal, i.e., free of dissection flap
+ - This portion of aorta lacks intima
+ - Sometimes called missing flap or naked aorta sign
+ - Anterograde IIIS can involve entire thoracic aorta and extend into abdominal aorta or into iliac arteries
+ - IIIS occluding superior mesenteric artery (SMA) can lead to mesenteric ischemia
+- ## MR Findings
+
+
+ - Few case reports of IIIS visualized with MR
+ - Best seen with bright-blood imaging, such as gated SSFP imaging or contrast-enhanced MRA
+ - Similar findings to CT with unidirectional or bidirectional intima that has rolled upon itself superiorly &/or inferiorly
+- ## Echocardiographic Findings
+
+
+ - May see portion of intimal flap prolapsing into LV
+ - 71% in retrograde IIIS
+ - Intimal flap in aortic root
+ - Aortic regurgitation; often severe
+ - 84% in retrograde IIIS
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - ECG-gated CTA
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Type A Dissection Without Intimointimal Intussusception
+
+
+ - Will not see delaminated intimal folded on itself in aortic lumen
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Anterograde
+ - Chest pain
+ - Neurologic symptoms (62%)
+ - Cerebrovascular accident
+ - Syncope
+ - Asymmetric blood pressure (57%)
+ - Likely due to subclavian artery occlusion
+ - Retrograde
+ - Chest and back pain
+ - Dyspnea
+ - Myocardial infarction (26.5%)
+ - Aortic regurgitation (84%); often severe
+- ## Demographics
+
+
+ - Mean age: 54 years
+ - M > F
+- ## Natural History & Prognosis
+
+
+ - Overall mortality: 24%
+ - Preoperative and postoperative mortality of 14% and 9%, respectively
+ - Similar mortality for anterograde and retrograde IIIS
+- ## Treatment
+
+
+ - Diagnosis not prospectively made before surgical intervention in 49% of anterograde IIIS and 16% of retrograde IIIS
+ - Aortotomy and surgical repair
+ - Various techniques, including Bentall repair, ascending aortic and arch replacement
+ - Intussuscepted intima may be retracted upward &/or downward and tacked down to aorta
+
+ 588886d4-5705-455d-83be-af8b469012b8
+
+## References
+
+# Selected References
+
+1. [Dokollari A et al: Aortic intimo-intimal intussusception in Stanford type A acute aortic dissection. Eur Heart J. 42(34):3410, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33085750%5Bpmid%5D)
+1. [Wu ZY et al: Aortic Intimo-intimal intussusception: a pooled analysis of published reports. Ann Vasc Surg. 75:471-8, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33831523%5Bpmid%5D)
+1. [Thunberg CA et al: Echocardiographic detection of intimo-intimal intussusception in a patient with acute Stanford type A aortic dissection. Ann Card Anaesth. 18(2):227-30, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25849697%5Bpmid%5D)
+1. [Sanders LH et al: Radiological diagnosis and classification of antegrade and retrograde Stanford type A intimal intussusception. Int J Cardiovasc Imaging. 23(5):659-65, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17160426%5Bpmid%5D)
+1. [Fan ZM et al: Acute aortic dissection with intimal intussusception: MRI appearances. AJR Am J Roentgenol. 186(3):841-3, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16498118%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Type A aortic dissection with bidirectional intimointimal intussusception (IIIS) is shown. At the point of circumferential intimal tear, the delaminated intima rolls anterograde into the aortic arch and retrograde into the left ventricle.*
+
+
+*Type A aortic dissection with bidirectional intimointimal intussusception (IIIS) is shown. At the point of circumferential intimal tear, the delaminated intima rolls anterograde into the aortic arch and retrograde into the left ventricle.*
+
+
+*Sagittal oblique CECT shows the anterograde IIIS folded upon itself in the proximal descending thoracic aorta . Intimal calcifications can be seen.*
+
+
+*Echo image during systole (left) shows a portion of the delaminated intima in the ascending aorta . During diastole (right), there is retrograde IIIS into the left ventricle .*
+
+
+*CTA in a 31-year-old man with chest and arm pain shows a type A dissection with a circumferential intimal tear and flap in the aortic root. The anterograde IIIS has lodged into and occluded the left subclavian artery . Only a small portion of intima is in the aortic arch .*
+
diff --git a/docs_md/articles/left-ventricular-enlargement_fbb972de-3e13-4c67-b7a4-f8901aa2efb8.md b/docs_md/articles/left-ventricular-enlargement_fbb972de-3e13-4c67-b7a4-f8901aa2efb8.md
new file mode 100644
index 0000000..9d77839
--- /dev/null
+++ b/docs_md/articles/left-ventricular-enlargement_fbb972de-3e13-4c67-b7a4-f8901aa2efb8.md
@@ -0,0 +1,197 @@
+---
+title: "Left Ventricular Enlargement"
+docid: "fbb972de-3e13-4c67-b7a4-f8901aa2efb8"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Left Ventricular Enlargement"
+ slug: "left-ventricular-enlargement"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "e8a63b4a-914d-475a-8dd1-cce5feaf7fec"
+imageCount: 15
+lastUpdated: "03/17/22"
+pageDescription: "Left Ventricular Enlargement"
+pageKeywords: "Cardiac, Differential Diagnosis, Left Ventricular Enlargement"
+pageTitle: "Left Ventricular Enlargement | STATdx"
+enhancedTitle: "Left Ventricular Enlargement"
+type: "DDX"
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Left Ventricular Enlargement"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Determination of LV chamber enlargement
+ - Radiographic
+ - Normal cardiothoracic ratio ≤ 0.5 on PA and ≤ 0.6 on AP at deep inspiration and proper positioning
+ - Expiratory and lordotic or rotated projections can change appearance of cardiac silhouette shape and size
+ - Leftward and downward displacement of left heart border
+ - LV extending 2 cm posterior to IVC border (Hoffman-Rigler sign) on lateral view
+ - Cross sectional
+ - LV volume may be measured qualitatively, not quantitatively, when only axial planes are available
+ - Reliable measurements require double oblique planes, usually short axis, and knowledge of phase within cardiac cycle
+ - Normal internal LV diameter at base is 3.9-5.3 cm for female and 4.2-5.9 cm for male patients
+ - 2-dimensional Simpson rule of discs in short axis or 3D auto-segmented are most reproducible
+ - Less reliable: Biplane method of Simpson rule and area length rule
+ - End-diastolic volume (EDV) > 170 mL in female and > 200 mL in male patients is indicative for enlargement
+ - EDV normalized by body surface area (EDV/BSA) are 2 standard deviations above mean if > 100 mL/m² in male and above 95 mL/m² in female patients
+ - Determination of LV wall thickness
+ - End-diastolic radial LV wall thickness > 1.2 cm is abnormal
+ - LV mass > 104 gm/m² in female or 119 gm/m² in male patients is specific for pathology
+ - Pitfalls
+ - Radiographic LV enlargement may be mimicked by pericardial effusion, expiration, poor lateral positioning or projection angle, or pericardial fat pad
+ - Misidentification of end diastole most frequent cause of erroneous left ventricular size measurement
+ - Cardiac volume may be affected by preimaging administration of β blockers or nitroglycerin
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Heart Failure**
+ - Ischemic cardiomyopathy most common etiology, followed by diabetes and hypertension
+ - EF < 40%
+ - Multivessel coronary artery calcifications or stenosis
+ - Evidence of prior infarct, subendocardial fat
+ - If retrospective gated CT or MR performed, myocardium can be evaluated for evidence of hibernation
+ - Subendocardial or transmural delayed enhancement present in coronary artery distribution indicates ischemia
+ - If delayed enhancement excludes subendocardial layer, nonischemic etiologies should be considered
+ - **Aortic Regurgitation**
+ - Bicuspid valve or calcified aortic valve
+ - Incomplete coaptation of cusps during diastole
+ - Regurgitant jet present on bright-blood MR
+ - **Mitral Regurgitation**
+ - Mitral valve calcifications
+ - Dilated left atrium
+ - Isolated right upper lobe edema is rare manifestation resulting from regurgitant jet
+ - **Acute M****yocardial Infarction**
+ - Enlarged cardiac silhouette compared to recent prior
+ - Supporting clinical information, troponin leak, ECG changes, or typical chest pain
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Patent Ductus Arteriosus**
+ - Initially, enlarged main pulmonary arteries; later, LV, LA, and ascending aortic enlargement
+ - LV enlargement with dilated ascending aorta in absence of valvular disease
+ - Best seen in gated CT or 3D MRA
+ - MR Qp:Qs ratio < 1:1
+ - **Coarctation of Aorta**
+ - Associated with bicuspid valve
+ - Hemodynamic narrowing represented by dilated intercostal collaterals
+ - Not to be confused with pseudocoarctation (tortuous arch without hemodynamic narrowing)
+ - Undiagnosed cases in adults often occur when narrowing distal to left subclavian take-off
+ - **Idiopathic Dilated Cardiomyopathy**
+ - Patients often < 60 years of age
+ - Diagnosis of exclusion
+ - Significant coronary artery occlusion or myocarditis to be excluded
+ - MR delayed enhancement present in ~ 40% of cases, most commonly mid-myocardial
+ - EF < 40% &/or fractional shortening < 25%
+ - **Hypertrophic Cardiomyopathy**
+ - LVOT view shows MR with systolic anterior motion of mitral valve leaflet
+ - Asymmetric septal, apical, and concentric variants exist
+ - In concentric variant, differential includes hypertensive heart disease/aortic stenosis, amyloidosis, and sarcoidosis
+ - Patchy mid myocardial enhancement in areas of LV thickening and RV insertion into LV
+ - **Amyloidosis**
+ - Patients typically > 65 years of age
+ - Increased LV wall thickness with poor or normal contractility
+ - Diffuse subendocardial perfusion defect
+ - Delayed enhancement inversion recovery sequences show equal relaxation times between blood pool and myocardium
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Athlete's Heart**
+ - Occurs in athletes who engage in prolonged aerobic activity
+ - End-diastolic wall thickness > 15 mm in young patient with dilated heart can be seen in athlete's heart
+ - LV volume will decrease following 3 months of deconditioning
+ - **Pregnancy-Induced Dilated Cardiomyopathy**
+ - Postpartum LV enlargement and hypokinesis
+ - Follow-up imaging in 3 months may show resolution
+ - **Alcohol-Induced Dilated Cardiomyopathy**
+ - Accompanying clinical history
+ - Follow-up imaging will show resolution if acute
+
+
+## Images
+
+
+### Selected Images
+
+
+**Heart Failure**
+*Coronal oblique NECT of ischemic heart failure shows LV enlargement with subepicardial fat , predominantly in an LAD distribution, representing prior infract.*
+
+
+**Heart Failure**
+*Coronal oblique NECT of ischemic heart failure shows LV enlargement with subepicardial fat , predominantly in an LAD distribution, representing prior infract.*
+
+
+**Heart Failure**
+*Short-axis inversion recovery MR through the LV mid-chamber shows dilated LV with late enhancement in a LAD distribution , compatible with ischemic cardiomyopathy.*
+
+
+**Heart Failure**
+*Four-chamber bright-blood MR in a patient with history of long, uncontrolled, standing hypertension shows a mildly dilated LV with diffuse wall thickening. This will eventually progress to an appearance indistinguishable from other dilated CM.*
+
+
+**Heart Failure**
+*Diastolic phase LVOT CECT shows markedly dilated LV without aortic valve disease. This patient had depressed EF and densely calcified coronary arteries, indicating ischemic cardiomyopathy.*
+
+
+**Aortic Regurgitation**
+*Coronal cine MR shows a turbulent jet originating at the aortic valve, directed toward the LV chamber .*
+
+
+**Mitral Regurgitation**
+*Systolic phase LVOT cine MR of mitral regurgitation shows low signal corresponding to regurgitation due to mitral valve prolapse. The prolapsing leaflet is seen with a regurgitant jet directed at the septum.*
+
+
+**Acute Myocardial Infarction**
+*Short-axis inversion recovery FSE MR through the LV mid-chamber shows mid-myocardial LAD distribution late enhancement . Hypointense subendocardium indicates acute MI associated microvascular obstruction .*
+
+
+**Patent Ductus Arteriosus**
+*Four-chamber CTA shows dilation of the left atrium and left ventricle from chronic volume overload due to left to right shunting across the patent ductus arteriosus (not shown).*
+
+
+**Patent Ductus Arteriosus**
+*Axial oblique CTA shows a connection between the proximal descending aorta and the pulmonary artery, diagnostic of a patent ductus arteriosus. Left-to-right shunt resulted in LV enlargement.*
+
+
+**Coarctation of Aorta**
+*Sagittal T1 C+ FS MR shows focal narrowing distal to the left subclavian take-off . Presence of intercostal collaterals and LV enlargement indicated a hemodynamically significant stenosis, differentiating it from pseudocoarctation.*
+
+
+**Dilated Cardiomyopathy**
+*Axial NECT in a 41-year-old man with symptoms of heart failure shows LV dilation without CAD. Cardiomyopathy etiology was not found, and a diagnosis of idiopathic dilated cardiomyopathy was made.*
+
+
+**Dilated Cardiomyopathy**
+*Short-axis inversion recovery FSE MR shows septal mid-myocardial enhancement in a patient with dilated cardiomyopathy .*
+
+
+**Hypertrophic Cardiomyopathy**
+*Diastolic phase LVOT bright-blood cine MR of asymmetric variant hypertrophic cardiomyopathy shows asymmetric thickening of interventricular septum at base . Study should be interrogated for fibrosis and SAM.*
+
+
+**Amyloidosis**
+*Short-axis inversion recovery FSE MR through LV mid-chamber 10 minutes post contrast shows near-equal relaxation of blood pool and myocardium. This finding is caused by altered contrast concentration kinetics due to presence of amyloid protein.*
+
+
+### Additional Images
+
+
+**Aortic Regurgitation**
+*Diastolic phase LVOT bright-blood cine MR of aortic regurgitation shows a turbulent jet originating at the aortic valve, directed toward the LV chamber .*
+
diff --git a/docs_md/articles/limited-intimal-tear_bb253de9-ab48-4740-89bd-0036fb8c12f5.md b/docs_md/articles/limited-intimal-tear_bb253de9-ab48-4740-89bd-0036fb8c12f5.md
new file mode 100644
index 0000000..803f76c
--- /dev/null
+++ b/docs_md/articles/limited-intimal-tear_bb253de9-ab48-4740-89bd-0036fb8c12f5.md
@@ -0,0 +1,311 @@
+---
+title: "Limited Intimal Tear"
+docid: "bb253de9-ab48-4740-89bd-0036fb8c12f5"
+authors:
+ - key: "5de0df07-7b3e-4678-8767-1519e1153f29"
+ value: "Dominik Fleischmann, MD"
+ - key: "5a4d7c03-82a7-4740-947a-9638213aec4a"
+ value: "Mohammad H. Madani, MD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Limited Intimal Tear"
+ slug: "limited-intimal-tear"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "99a74e38-6a58-4b21-94d4-8e0da4c2bf13"
+imageCount: 17
+lastUpdated: "02/10/25"
+pageDescription: "Limited Intimal Tear"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Limited Intimal Tear"
+pageTitle: "Limited Intimal Tear | STATdx"
+enhancedTitle: "Limited Intimal Tear"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Limited Intimal Tear"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Limited tear, incomplete dissection, partial thickness tear, limited dissection
+ - Limited tears of aorta are rare cause of acute aortic syndromes (AASs), representing ~ 5% of AASs
+- ## Imaging
+
+
+ - In general, limited intimal tears (LITs) appear as intimal irregularity or defect, often associated with undermined edge (reminiscent of localized dissection), which may contain small amount of thrombus, and bulging of corresponding outer wall of aorta
+ - Actual **tear**can be subtle and difficult to delineate, as it can be linear or have more complex shapes
+ - **Edges** of tear can be subtle, unexpected contour irregularity of inner surface of aorta; edges can be lifted or rolled off remainder of wall, reminiscent of dissection; undermined edges can contain intramural thrombus
+- ## Clinical Issues
+
+
+ - **Natural history** of acute LITs is poorly understood
+ - LITs are **treated similar to aortic dissections**
+ - Stanford type A LITs (affecting ascending aorta) typically undergo open surgical replacement of aorta
+ - In Stanford type B LITs (not involving ascending aorta), medical management is appropriate unless complications occur; anatomically suitable type B LITs can undergo endovascular aortic repair
+ - All patients with LITs require **life-long follow-up** and imaging surveillance
+- ## Diagnostic Checklist
+
+
+ - Key to diagnosing LITs is being aware of existence of these relatively rare lesions
+ - Contour irregularities of inner surface of aorta, which cannot be explained by motion artifacts or other cause, should raise suspicion of LIT
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Limited intimal tear (LIT)
+- ## Synonyms
+
+
+ - Limited tear, incomplete dissection, partial thickness tear, limited dissection
+- ## Definitions
+
+
+ - Limited tears of aorta are rare cause of acute aortic syndromes (AASs), representing ~ 5% of AASs
+ - Limited tears fall under spectrum of diseases characterized pathologically by degeneration of media layer of aortic wall
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Intimal irregularity or defect, often associated with undermined edge, which may contain small amount of thrombus; bulging of delaminated and exposed outer wall of aorta
+ - ### Location
+
+
+ - LITs occur more commonly in ascending aorta than in arch or descending aorta
+ - LITs are oriented longitudinally, or are circumferentially relative to aortic axis
+ - ### Size
+
+
+ - Tear itself can range from few millimeters to several centimeters in length; width can range from few mm to few centimeters
+ - ### Morphology
+
+
+ - Actual tear can be subtle and difficult to delineate
+ - Can be linear or have more complex shapes (figure 8 shape)
+ - Edges of tear can be subtle
+ - Can appear as unexpected contour irregularity of inner surface of aorta
+ - Edges can also be undermined with portion of intimomedial tissue lifted off remainder of wall, reminiscent of dissection, but without fully formed false lumen
+ - Outer wall of aorta at level of LIT may show subtle focal bulging
+ - Outer wall of aorta only consists of residual adventitia, equivalent to outer wall of false lumen in aortic dissection
+- ## CT Findings
+
+
+ - Aortic luminal contour abnormality in shape of linear or complex-shaped tear
+ - Edge of tear is better visualized if it is lifted off, giving appearance of focal dissection flap, or accompanied by small amount of intramural thrombus
+ - Exposed and delaminated remainder of (outer) aortic wall bulges outward
+ - Associated findings may be similar to those seen in aortic dissection
+ - Pericardial fluid or hemopericardium (in type A lesions)
+ - Pulmonary artery subadventitial hematoma
+ - Periaortic mediastinal stranding (in type B lesions)
+- ## Ultrasonographic Findings
+
+
+ - LITs have been described on transesophageal and transthoracic echocardiography
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - CTA, ideally with ECG gating
+ - ### Protocol advice
+
+
+ - CT scans without motion artifacts in ascending aorta can be achieved with fast scanning techniques (fast gantry rotation, high-pitch, wide detector), and, most reliably, by ECG gating
+ - If ECG gating is not routinely performed, or if suspicious aortic lesion is detected (e.g., on pulmonary embolism CT study), repeat injection and ECG gated scan can be performed
+ - Multiplanar reformations are essential and increase confidence for presence or absence of subtle LIT
+ - 3D volume-rendered images with "transparent blood" display are most helpful to see shape and extent of lesion
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Artifacts
+
+
+ - Motion artifacts from transmitted cardiac pulsation (double contours of aortic wall on CT) can simulate or obscure subtle LITs
+ - Motion artifacts are often visible on both opposing aortic walls, whereas true aortic lesions usually affect only 1 side
+ - Repeat scan with ECG gating usually allows accurate diagnosis
+- [Intramural Hematoma](/document/aortic-intramural-hematoma/128bc4cc-a26d-47d5-90e7-b1a1f608e657)
+ - There is overlap between spectrum of LITs and spectrum of intramural hematomas (IMHs)
+ - LITs can be associated with localized intramural blood, notably at undermined edges of tear, but predominant finding is intimal tear and defect
+ - It would not be fundamentally wrong to consider some LITs as IMH with large entry tears
+ - Main task for radiologist is to recognize lesion as acute aortic pathology
+ - Treatment is dictated by location and presence of complications rather than by specific type or classification of lesion
+- [Penetrating Atherosclerotic Ulcer](/document/penetrating-atherosclerotic-ulcer/63f6cba2-2200-456a-8d03-ac3111e420c8)
+ - LITs are sometimes mislabeled as PAUs, most notably if LIT is small and has small hematoma associated with it
+ - Pathology of PAUs is very different though, since PAUs are atherosclerotic lesions, whereas LITs are under spectrum of diseases characterized by media degeneration
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - LITs fall under spectrum of aortic diseases characterized pathologically be degeneration of aortic media
+ - Formerly, but incorrectly, termed cystic media necrosis
+ - Other diseases characterized by media degeneration are classic aortic dissection and IMH (dissection variant)
+ - Of note, media degeneration can precede acute event for years; loss of coherence between layers of aortic wall can go unnoticed for long time until entry tear allows physical separation and delamination of wall layers
+ - Media degeneration is prerequisite for aortic dissection and its variants, IMH, and LIT; conversely, normal aorta does not dissect
+ - Causes of media degeneration are
+ - Severe, untreated hypertension
+ - Most common cause of media degeneration
+ - Aging: Normal aging results in media degeneration
+ - Genetic diseases: Marfan syndrome, Ehlers-Danlos IV syndrome, familial aortic aneurysms and dissections
+- ## Staging, Grading, & Classification
+
+
+ - Limited tears are anatomically categorized similar to aortic dissections
+ - Stanford type A for ascending aortic lesions
+ - Stanford type B for all other lesions that do not affect ascending aorta
+ - Similar to classic dissection, LITs are considered hyperacute (24 hours), acute (< 14 days), subacute, or chronic (> 3 months)
+- ## Gross Pathologic & Surgical Features
+
+
+ - Intimal-medial tear; limited or focal medial layer dissection plane, medial degeneration
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Clinical presentation of acute LITs is similar to other acute diseases of aorta, summarized under clinical term acute AASs
+ - AAS symptoms: Acute, sharp, severe chest or back pain
+ - AAS can be caused by number of aortic diseases other than LITs, such as classic dissection, IMH, PAU, and also rupturing aneurysms
+ - ### Other signs/symptoms
+
+
+ - LITs may cause little or no symptoms, evidenced by occasional incidental detection of chronic LITs in patients who do not remember specific event
+- ## Demographics
+
+
+ - Patients with LITs are on average slightly older than patients with classic aortic dissection and patients with IMH
+- ## Natural History & Prognosis
+
+
+ - Natural history of acute LITs is poorly understood
+ - **Acute phase**: Similar to aortic dissection, first 14 days are considered acute phase of disease
+ - **Subacute phase** (15-90 days): LITs seem to evolve with any IMH component receding and disclosing depth of undermined edges; tear may become more visible if edges become thicker and less mobile, and exposed adventitial bulge may become deeper (similar to false lumen increase during subacute phase in dissection)
+ - **Chronic phase**: Delaminated portion of aortic wall that is exposed to systemic pressure will continue to dilate and can become aneurysmal; since less wall is exposed in LITs than in classic dissection, changes over time may be less
+ - Chronic LITs are occasionally detected in asymptomatic patients, suggesting that they may present with less conspicuous symptoms in some cases
+ - All patients with LITs require life-long follow-up and imaging surveillance
+- ## Treatment
+
+
+ - LITs are treated similar to aortic dissections
+ - Stanford type A LITs (affecting ascending aorta) typically undergo open surgical replacement of aorta
+ - In Stanford type B LITs (not involving ascending aorta), medical management is appropriate unless complications occur, such as rupture and malperfusion
+ - Anatomically suitable type B LITs requiring intervention will undergo thoracic endovascular aortic repair
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - Key to diagnosing LITs is being aware of existence of these relatively rare lesions
+- ## Image Interpretation Pearls
+
+
+ - Contour irregularities of inner surface of aorta, which cannot be explained by motion artifacts or other causes, should raise suspicion of LIT
+ - If associated with undermined edge or with subtle intramural blood, this should prompt further visualization with multiplanar reformats, and, ideally, with volume-rendered 3D images, which can more clearly demonstrate characteristics of this lesion
+- ## Reporting Tips
+
+
+ - LITs are aortic emergency and need to be reported and communicated urgently, similar to acute aortic dissection
+ - Distinction between type A lesions (ascending aorta involved) vs. type B lesions is critical, since former usually undergo urgent surgical repair
+ - Presence of complications, such as pericardial fluid, hemopericardium, tamponade; signs of rupture, branch vessel compromise
+
+ 69fc13cf-566f-40e2-821b-39b63a2d0a8d
+
+## References
+
+# Selected References
+
+1. [Madani MH et al: Limited aortic intimal tears: CT imaging features and clinical characteristics. Radiol Cardiothorac Imaging. 4(6):e220155, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36601454%5Bpmid%5D)
+1. [Chin AS et al: Acute limited intimal tears of the thoracic aorta. J Am Coll Cardiol. 71(24):2773-85, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29903350%5Bpmid%5D)
+1. [Svensson LG et al: Intimal tear without hematoma: an important variant of aortic dissection that can elude current imaging techniques. Circulation. 99(10):1331-6, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10077517%5Bpmid%5D)
+1. [Murray CA et al: Spontaneous laceration of ascending aorta. Circulation. 47(4):848-58, 1973](http://www.ncbi.nlm.nih.gov/pubmed/?term=4696804%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*CT images (top) show a linear filling defect and contour irregularity in the ascending and transverse aorta. Note undermined edges of the tear and the bulging wall . 3D images (bottom) show a longitudinally oriented limited tear seen from the outside (left) and inside (right). (Courtesy Chin et al.)*
+
+
+*CT images (top) show a linear filling defect and contour irregularity in the ascending and transverse aorta. Note undermined edges of the tear and the bulging wall . 3D images (bottom) show a longitudinally oriented limited tear seen from the outside (left) and inside (right). (Courtesy Chin et al.)*
+
+
+*Axial ECG gated images of a limited intimal tear (LIT) show contour irregularities in the ascending aorta, which correspond to the undermined edges of a large LIT. (Courtesy Chin et al.)*
+
+
+*Schematic shows the luminal view of intimomedial tear shapes (column 1), their stretched open luminal appearance in a pressurized aorta (column 2), external bulges (column 3), and side view (column 4). Schematic also illustrates linear (A), L-shaped (B), T-shaped (C), and star/complex shaped (D) tears. (Courtesy Madani et al.)*
+
+
+*Inner/luminal view of a semicircumferential LIT in the ascending aorta is shown. Note that the tear is stretched open in vivo (left). The edge of the tear is viewed from above (right).*
+
+
+*External view of the semicircumferential LIT shows a band or stripe of bulging tissue in the ascending aorta with sharp, undermined edges .*
+
+
+*Corresponding intraoperative photograph shows a very thin aortic wall only consisting of residual adventitia. The normal aortic wall is thicker with a more yellow hue due to the underlying intact media, which contains elastin.*
+
+
+*Photograph of the LIT in a resected aortic wall is shown. Note that in vitro, the shape of the tear is mostly linear with a small "L" on the right end. In vivo, in a pressurized aorta, that tear appears stretched open, and exposed underlying adventitial will bulge out (as shown on CT images).*
+
+
+*CECT shows contour abnormality with a filling defect representing a limited aortic intimal tear . An associated periaortic and mediastinal hematoma is consistent with impending rupture.*
+
+
+*The proximal end of the LIT shows the undermined edge, reminiscent of a dissection, seen on the left aspect of the ascending thoracic aorta . (Courtesy S. Kligerman, MD.)*
+
+
+*Chronic LITs show slightly thicker , undermined edges, no periaortic stranding, and no associated intramural blood.*
+
+
+*Corresponding oblique sagittal CECT in the same patient shows a focal tissue flap consistent with an undermined edge of a LIT . (Courtesy S. Kligerman, MD.)*
+
+
+*The classic appearance of a LIT in the aortic arch with a wide, stretched open tear with undermined edges and bulging of the outer aortic wall is shown.*
+
+
+*Corresponding oblique coronal view shows a LIT with a small, undermined edge/flap and a mild bulge of the outer aortic contour . (Courtesy S. Kligerman, MD.)*
+
+
+*Very subtle contour irregularity in the proximal descending thoracic aorta is seen, which was initially missed on day 1 of hospitalization when only axial images were reviewed.*
+
+
+*Follow-up imaging (day 6) again shows only very subtle bulging of the descending thoracic aorta with contour irregularities at the edge . Only the side-by-side comparison raises the suspicion of an aortic abnormality.*
+
+
+*3D volume-rendered view of the proximal descending aorta clearly shows the LIT is apparent even on day 1.*
+
+
+*3D volume-rendered image on day 6 clearly shows the LIT and that it has grown and expanded over just a few days.*
+
diff --git a/docs_md/articles/marfan-syndrome_61d06223-8428-401c-89bc-d12205410726.md b/docs_md/articles/marfan-syndrome_61d06223-8428-401c-89bc-d12205410726.md
new file mode 100644
index 0000000..233e451
--- /dev/null
+++ b/docs_md/articles/marfan-syndrome_61d06223-8428-401c-89bc-d12205410726.md
@@ -0,0 +1,418 @@
+---
+title: "Marfan Syndrome"
+docid: "61d06223-8428-401c-89bc-d12205410726"
+authors:
+ - key: "9fea2857-d729-4fe4-b4fd-3b7bf1db23cf"
+ value: "Mortadha Al-Kinani, MD, MBChB"
+ - key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
+ value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Marfan Syndrome"
+ slug: "marfan-syndrome"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "ccb5aa34-99dc-4558-9576-462716224003"
+imageCount: 30
+lastUpdated: "01/28/25"
+pageDescription: "Marfan Syndrome"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Marfan Syndrome"
+pageTitle: "Marfan Syndrome | STATdx"
+enhancedTitle: "Marfan Syndrome"
+type: "DX"
+references: true
+ddx: true
+cases: 1
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Marfan Syndrome"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Congenital systemic connective tissue disorder; skeletal, cardiovascular, and ocular abnormalities
+- ## Imaging
+
+
+ - Radiography
+ - Ascending aortic aneurysm
+ - Cardiomegaly
+ - Pectus deformity, scoliosis, scalloped vertebrae
+ - Pneumothorax, apical bullae
+ - CT
+ - Annuloaortic ectasia, aneurysm
+ - Aortic rupture: Crescent sign, hematoma
+ - Dissection: Intimal flap, true/false lumen
+ - Echocardiography
+ - At diagnosis to assess ascending aorta and 6 months thereafter to determine rate of enlargement
+ - MR: Similar to CT in sensitivity
+- ## Top Differential Diagnoses
+
+
+ - Familial thoracic aortic aneurysm
+ - Ehlers-Danlos syndrome
+ - Bicuspid aortic valve
+- ## Pathology
+
+
+ - Mutation in *FBN1* gene encoding for fibrillin 1
+ - Autosomal dominant; 25% de novo mutations
+ - Microscopy: Cystic medial necrosis
+- ## Clinical Issues
+
+
+ - Cardiac/vascular abnormalities
+ - Pulmonary abnormalities
+ - Thoracic skeletal abnormalities
+- ## Diagnostic Checklist
+
+
+ - Consider Marfan syndrome in young patients with ascending aortic aneurysm &/or aortic dissection
+
+# TERMINOLOGY
+
+- ## Definitions
+
+
+ - Marfan syndrome (MFS): Congenital systemic connective tissue disorder characterized by **skeletal**, **cardiovascular**, and **ocular** abnormalities
+
+# IMAGING
+
+- ## Radiographic Findings
+
+
+ - **Ascending aortic aneurysm**: Mediastinal widening, right superior cardiomediastinal contour abnormality
+ - **Cardiomegaly**: Aortic/mitral regurgitation, cardiomyopathy
+ - Pectus deformity (excavatum, carinatum), scoliosis, scalloped vertebral bodies
+ - Pneumothorax, apical bullae
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - **Annuloaortic ectasia****/****aneurysm**: Effacement of sinotubular junction (60-80% of patients)
+ - Lack of normal transition between aortic root and tubular portion of ascending aorta
+ - Indication for surgery: Diameter > 4.5 cm
+ - **Aortic rupture**, often contained
+ - **Crescent sign**: Crescentic eccentric aortic high-attenuation area
+ - **Hematoma**: Mediastinal high attenuation, hemothorax, hemopericardium
+ - ### CTA
+
+
+ - More sensitive than radiography
+ - Direct visualization
+ - Dissection: Intimomedial defect, true/false lumen
+ - Rupture: Active extravasation
+- ## MR Findings
+
+
+ - Equivalent to CT, similar accuracy
+ - Direct visualization of aortic aneurysm and dissection
+ - Cine MR and phase-contrast imaging are optimal for valve assessment: Aortic &/or mitral regurgitation
+ - 4D flow MRA provides detailed hemodynamic assessments, including pulse wave velocity (PWV) and peak systolic velocity, to predict future aortic events
+- ## Echocardiographic Findings
+
+
+ - Initial assessment of aortic size and 6 months thereafter to determine rate of enlargement
+- ## Imaging Recommendations
+
+
+ - ### Protocol advice
+
+
+ - Annual imaging is recommended after initial echocardiography
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Familial Thoracic Aortic Aneurysm
+
+
+ - Sinus of Valsalva aortic aneurysm
+- ## Ehlers-Danlos Syndrome
+
+
+ - Aneurysm/rupture: Medium/large muscular arteries
+ - Systemic: Joint hypermobility, atrophic scars, easy bruising, hernias, hollow organ rupture
+- [Bicuspid Aortic Valve](/document/bicuspid-aortic-valve/4cdfc5fe-0ac0-4969-b68b-6e9941c566f6)
+ - Ascending aortic aneurysm; bicuspid aortic valve
+- ## Homocystinuria
+
+
+ - Cardiac: Mitral valve prolapse
+ - Vascular: Intravascular thrombosis
+ - Systemic: Tall stature, ectopia lentis, long bone overgrowth, intellectual disability
+- ## Loeys-Dietz Syndrome
+
+
+ - Cardiac: Patent ductus arteriosus, atrial septal defect, bicuspid aortic valve
+ - Vascular: Sinus of Valsalva aneurysm, arterial tortuosity, aneurysms in other arteries
+ - Hypertelorism, cleft palate, broad or bifid uvula, exotropia, craniosynostosis, malar hypoplasia, blue sclerae, dolichostenomelia, arachnodactyly, pectus deformity, scoliosis, joint laxity, rare developmental delay
+- ## Shprintzen-Goldberg Syndrome
+
+
+ - Vascular: Rare sinus of Valsalva aneurysm
+ - Systemic: Hypertelorism, craniosynostosis, arched palate, arachnodactyly, pectus deformity, scoliosis, joint laxity, developmental delay
+- ## MASS (Mitral, Aortic, Skin, and Skeletal Manifestations) Syndrome
+
+
+ - Cardiac: Mitral valve prolapse
+ - Vascular: Borderline/nonprogressive aortic root enlargement
+ - Systemic: Nonspecific skin and skeletal findings, myopia
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Mutation in *FBN1*gene encoding for **fibrillin 1**
+ - *TGFBR2* and *TGFBR1* responsible for 10% of all cases
+ - ### Genetics
+
+
+ - Autosomal dominant; 25% de novo mutations
+- ## Microscopic Features
+
+
+ - **Cystic medial necrosis**
+ - Accumulation of basophilic ground substance in media with cyst-like lesions; no overt cystic or necrotic changes are normally identified
+ - Can occur in MFS, Ehlers-Danlos syndrome, and annuloaortic ectasia
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Clinical profile
+
+
+ - **Cardiac abnormalities**
+ - Mitral valve regurgitation
+ - Children: Mitral regurgitation and heart failure, pulmonary hypertension, death in infancy
+ - > 50% auscultatory/echocardiographic evidence of mitral valve dysfunction, typically prolapse
+ - Progression of mitral valve prolapse to mitral regurgitation by adulthood
+ - Aortic valve regurgitation: Late occurrence from aortic anulus stretching
+ - Tricuspid valve prolapse
+ - Dilated cardiomyopathy (uncommon)
+ - **Vascular abnormalities**: Most common life-threatening manifestations
+ - **Annuloaortic ectasia**and **aortic aneurysm**
+ - **Aortic dissection**
+ - Often type A
+ - Acute-onset heart failure typically from severe aortic insufficiency
+ - Extension to coronary arteries; myocardial infarction or sudden cardiac death
+ - Dilatation/dissection of descending thoracic/abdominal aorta
+ - Dilatation of pulmonary trunk
+ - **Pulmonary abnormalities**
+ - Bullae: Predisposed to spontaneous pneumothorax
+ - **Thoracic skeletal abnormalities**
+ - Pectus deformity; can contribute to restrictive lung disease
+ - **Revised Ghent nosology (Ghent 2)**
+ - **Goals**: Identification of patients with higher risk for aortic aneurysm or dissection; simplicity of use of diagnostic criteria; allowance for early diagnosis; consideration of availability and costs of diagnostic tests; better definition of entities, such as familial ectopia lentis, MASS phenotype, and mitral valve prolapse syndrome; and delineation of triggers for alternative diagnoses, such as Loeys-Dietz syndrome
+ - **5 major changes** (in comparison with Ghent 1)
+ - More diagnostic emphasis on aortic root aneurysm/dissection and ectopia lentis
+ - More prominent role of molecular genetic testing (i.e., *FBN1*; *TGFBR1* and *TGFBR2*)
+ - Complete removal of some clinical criteria (e.g., dilatation of main pulmonary artery, dilatation or dissection of descending thoracic or abdominal aorta, increased axial length of globe and abnormally flat cornea, hypoplastic iris or hypoplastic ciliary muscle causing decreased miosis, joint hypermobility, spondylolisthesis, highly arched palate, and recurrent or incisional hernia, calcification of mitral anulus, apical blebs of lung), or mitigation of diagnostic relevance of dural ectasia, or adding or modifying clinical criteria, such as myopia > 3 diopters, hindfoot valgus, and thoracolumbar kyphosis
+ - Provision of discriminating features of alternative diagnoses, such as Loeys-Dietz syndrome
+ - Provision of context-specific recommendations for patient counseling and follow-up
+ - **Absence of family history**
+ - Aortic root diameter (Z-score ≥ 2) and ectopia lentis = MFS
+ - Aortic root diameter (Z-score ≥ 2) and causal *FBN1*mutation = MFS
+ - Aortic root diameter (Z-score ≥ 2) and systemic score ≥ 7 points = MFS
+ - Ectopia lentis and causal *FBN1*mutation with known aortic root dilatation = MFS
+ - **In presence of family history**
+ - Ectopia lentis and family history of MFS = MFS
+ - Systemic score ≥ 7 points and family history of MFS = MFS
+ - Aortic root diameter (Z-score ≥ 2 above 20 years old, ≥ 3 below 20 years) and family history of MFS = MFS
+ - **Scoring of systemic features** (maximum total score 20 points)
+ - Wrist and thumb sign = 3 points (wrist or thumb sign = 1 point)
+ - Pectus carinatum deformity = 2 points (pectus excavatum or chest asymmetry = 1 point)
+ - Hindfoot deformity = 2 points (plain pes planus = 1 point)
+ - Protrusio acetabuli = 2 points
+ - Reduced upper segment:lower body segment ratio and increased arm/height and no severe scoliosis = 1 point
+ - Scoliosis or thoracolumbar kyphosis = 1 point
+ - Reduced elbow extension = 1 point
+ - Facial features (3/5) = 1 point (dolichocephaly, enophthalmos, downslanting palpebral fissures, malar hypoplasia, retrognathia)
+ - Pneumothorax = 2 points
+ - Skin striae = 1 point
+ - Myopia > 3 diopters = 1 point
+ - Mitral valve prolapse (all types) = 1 point
+ - Dural ectasia = 2 points
+- ## Demographics
+
+
+ - ### Sex
+
+
+ - M = F
+ - ### Epidemiology
+
+
+ - Incidence: 1 in 3,000-5,000 individuals
+- ## Natural History & Prognosis
+
+
+ - Improved prognosis with annual imaging, medical/surgical intervention
+ - Higher aortic dissection risk during pregnancy
+- ## Treatment
+
+
+ - β-adrenergic receptor blockade
+ - Restriction of vigorous exercise
+ - Surgical reconstruction: Elective according to aortic diameter or if dissection or rupture
+ - Indications for surgery
+ - Ascending aorta
+ - Diameter ≥ 4.5 cm
+ - Diameter ≥ 4.5 cm in family history of early dissection, rapid growth defined as ≥ 0.5 cm/year, and significant aortic regurgitation
+ - Aortic arch
+ - Diameter > 5.0 cm
+ - Descending thoracic aorta
+ - Diameter > 5.0 cm
+
+ 1538f38d-6569-48a2-831a-03ab0378e5be
+
+## References
+
+# Selected References
+
+1. [Brownstein AJ et al: Genes associated with thoracic aortic aneurysm and dissection: 2018 update and clinical implications. Aorta (Stamford). 6(1):13-20, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30079932%5Bpmid%5D)
+1. [Groner LK et al: Imaging of the postsurgical aorta in Marfan syndrome. Curr Treat Options Cardiovasc Med. 20(10):80, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30146656%5Bpmid%5D)
+1. [Geiger J et al: Longitudinal evaluation of aortic hemodynamics in Marfan syndrome: new insights from a 4D flow cardiovascular magnetic resonance multi-year follow-up study. J Cardiovasc Magn Reson. 19(1):33, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28327193%5Bpmid%5D)
+1. [von Kodolitsch Y et al: Perspectives on the revised Ghent criteria for the diagnosis of Marfan syndrome. Appl Clin Genet. 8:137-55, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26124674%5Bpmid%5D)
+1. [Hiratzka LF et al: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 122(4):e410, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20233780%5Bpmid%5D)
+1. [Loeys BL et al: The revised Ghent nosology for the Marfan syndrome. J Med Genet. 47(7):476-85, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20591885%5Bpmid%5D)
+1. [Ha HI et al: Imaging of Marfan syndrome: multisystemic manifestations. Radiographics. 27(4):989-1004, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17620463%5Bpmid%5D)
+1. [De Backer JF et al: Primary impairment of left ventricular function in Marfan syndrome. Int J Cardiol.112(3):353-8, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16316698%5Bpmid%5D)
+1. [Boileau C et al: Molecular genetics of Marfan syndrome. Curr Opin Cardiol. 20(3):194-200, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15861007%5Bpmid%5D)
+1. [Judge DP et al: Marfan's syndrome. Lancet. 366(9501):1965-76, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16325700%5Bpmid%5D)
+1. [Meijboom LJ et al: Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J. 26(9):914-20, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15681576%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Dilated Aorta
+DDX:9daee273-f1e9-4cf9-a979-8990a9b82e40
+
+## Cases
+
+- {'cases': [{'authors': [{'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}, {'key': '3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1', 'value': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI'}], 'caseVersionId': 'e3fd2ed7-6fcc-4541-bbfd-aac36ee456ab', 'description': "Oblique coronal volume-rendered (#1) and short axis (#2) contrast-enhanced CTAs show annuloaortic ectasia with "tulip bulb" or "pear-shaped" dilation of the aortic annulus (white arrows, #1), the sinus of Valsalva (black arrows), and the more distal ascending aorta (annuloaortic ectasia). \n\nSagittal oblique MIP (#3) demonstrates effacement of the sinotubular junction. There is superior displacement of the right coronary artery origin (open arrow), and scoliosis related to patient's underlying diagnosis of Marfan syndrome. \n\nAxial contrast-enhanced CT (#4) shows upper lung paraseptal and bullous emphysema (open black arrows), which is another common manifestation of Marfan syndrome, predisposing the patient to spontaneous pneumothoraces.", 'history': 'Marfan patient with history of ascending aortic aneurysm.', 'imagePoolId': 'ad500e5f-0216-481c-ac7b-34782bc7635e', 'name': 'Annuloaortic ectasia', 'teachingPoint': None, 'demographics': '43 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Graphic compares a normal ascending aorta (left) with a well-defined sinotubular junction and annuloaortic ectasia with sinotubular junction effacement (right).*
+
+
+*Graphic compares a normal ascending aorta (left) with a well-defined sinotubular junction and annuloaortic ectasia with sinotubular junction effacement (right).*
+
+
+*Coronal oblique CECT shows aneurysms associated with bicuspid aortic valve (left) and Marfan syndrome (MFS) (right) to exemplify annuloaortic ectasia. Note that a sinotubular junction is still identifiable in dilatation due to bicuspid aortic valve but is effaced in MFS due to dilated aortic root.*
+
+
+*PA radiograph of the chest in a patient with MFS shows an abnormal convexity along the right superior cardiac silhouette. This finding is often associated with a dilated ascending aorta. In young patients, it is commonly associated with bicuspid aortic valve and MFS.*
+
+
+*Axial chest CTA in the same patient shows dilatation of the sinuses of Valsalva (4.7.x 5.0 x 5.0 cm). Three measurements bisecting each sinus are usually obtained at this level.*
+
+
+*Axial CECT in a young patient with MFS shows symmetric dilation of all sinuses of Valsalva.*
+
+
+*Coronal CECT in the same patient shows effacement of the sinotubular junction , which is characteristic of MFS. Note diffuse dilatation of the entire ascending thoracic aorta, demonstrating a morphology often referred to as the tulip bulb sign. The pulmonary trunk appears smaller than the aorta. Normally, the pulmonary artery is of the same size as the ascending aorta.*
+
+
+*Coronal CECT 3D surface rendering in the same patient shows the so-called tulip bulb configuration of the ascending thoracic aorta characteristically seen in MFS due to dilatation of the aortic root with normal appearance of the arch and descending thoracic aorta.*
+
+
+*PA chest radiograph in a patient with MFS and acute aortic syndrome shows no specific cause of the symptoms reported in this individual. A normal radiograph does not exclude aortic dilatation or dissection.*
+
+
+*Axial CECT in the same patient shows marked aortic root dilatation . Note disproportion in the diameter of the pulmonary trunk, which should typically be the same size as the ascending aorta. At the higher level, there is an intimomedial defect representing ascending aortic dissection .*
+
+
+*Oblique sagittal CECT in the same patient shows the classic tulip bulb appearance due to annuloaortic ectasia.*
+
+
+*Axial CECT in a young pregnant woman with MFS with acute chest pain shows an intimomedial defect ascending into the descending aorta . Note dilation of the ascending thoracic aorta.*
+
+
+*Coronal oblique CECT in the same patient shows an intimomedial defect extending along the entire ascending aorta. Note effacement of the sinotubular junction , completely absent in this case, a very characteristic feature seen in MFS involving the ascending aorta.*
+
+
+*Sagittal oblique CECT in the same patient shows an extensive Stanford type A intimomedial defect involving the entire thoracic aorta. Dissection is a known complication of MFS, and pregnant individuals are under particular risk to develop such a complication.*
+
+
+*Axial chest CTA MIP reformation in a patient with MFS shows dilatation of the pulmonary trunk and central pulmonary arteries. (Courtesy L. Heyneman, MD.)*
+
+
+*Axial CTA in a patient with MFS shows marked dilatation of the aortic root and pectus excavatum deformity . These are major diagnostic criteria for MFS. (Courtesy L. Heyneman, MD.)*
+
+
+*Axial NECT in the same patient shows bilateral apical bullae . This may lead to secondary spontaneous pneumothorax in patients with MFS. (Courtesy L. Heyneman, MD.)*
+
+
+*Axial CECT in a patient with MFS who underwent a modified Bentall reconstruction of the ascending aorta shows pseudoaneurysm of the left coronary button .*
+
+
+*Coronal oblique CECT in the same patient shows an abnormal contract collection adjacent to the proximal aspect of the graft. Note distal anastomotic felts . Pseudoaneurysm of the coronary buttons is overall an uncommon complication that occurs more frequently in patients with MFS.*
+
+
+*Axial CECT in a patient with MFS who underwent a modified Bentall reconstruction of the aorta shows bulb-like dilatation of the left coronary button . Patients with MFS are more prone to develop dilation &/or pseudoaneurysm of the coronary buttons.*
+
+
+*Oblique sagittal SSFP MR in a patient with MFS and ascending aortic aneurysm shows a diastolic jet across the aortic valve, which constitutes one of the major cardiovascular criteria for MFS.*
+
+
+*Oblique gadolinium-enhanced MRA in the same patient shows dilatation of the aortic root with tulip bulb-like morphology. Volumetric acquisition and postprocessing reformations are some of the advantages of MRA over other sequences.*
+
+
+*Oblique sagittal SSFP and dark blood (HASTE) MR in a patient with MFS show dilatation of the proximal ascending aorta with effacement of the sinotubular junction. Both sequences are suitable to depict ascending aortic morphology.*
+
+
+### Additional Images
+
+
+*AP chest radiograph in a patient with MFS shows enlargement of the ascending aorta and cardiomegaly.*
+
+
+*PA chest radiograph in a patient with MFS shows an elongated thorax and dilatation of the ascending and descending aorta.*
+
+
+*Coronal CTA in the same patient shows typical findings of annuloaortic ectasia with obliteration of the sinotubular ridge and dilatation of the ascending aorta and the sinuses of Valsalva.*
+
+
+*Lateral chest radiograph in the same patient shows dilatation of the ascending aorta from annuloaortic ectasia.*
+
+
+*Coronal CECT in the same patient shows annuloaortic ectasia and an ascending aortic dissection with a visible intimal flap .*
+
+
+*Axial CECT in the same patient shows an ascending aorta dissection with a visible intimal flap . Note dural ectasia manifesting with scalloping of the vertebral body .*
+
+
+*DSA of the intracranial arteries shows the intracranial carotid artery as being markedly elongated, dilated, and redundant in the supraclinoid segment . Patients with MFS may have aneurysms of the extracranial and intracranial carotid arteries. Redundancy in carotid arteries may be associated with dissection.*
+
+
+*DSA shows marked redundancy of the vertebral artery . The redundancy is due to the structural abnormalities of the artery.*
+
diff --git a/docs_md/articles/mycotic-aneurysm_20616d0a-7e0b-48d9-9be9-1af29e3dd6da.md b/docs_md/articles/mycotic-aneurysm_20616d0a-7e0b-48d9-9be9-1af29e3dd6da.md
new file mode 100644
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+++ b/docs_md/articles/mycotic-aneurysm_20616d0a-7e0b-48d9-9be9-1af29e3dd6da.md
@@ -0,0 +1,468 @@
+---
+title: "Mycotic Aneurysm"
+docid: "20616d0a-7e0b-48d9-9be9-1af29e3dd6da"
+authors:
+ - key: "ee6ece9d-ad74-458c-a8df-11628ae7f879"
+ value: "Arzu Canan, MD"
+ - key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
+ value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
+ - key: "501b57b9-1723-4b1e-b21e-d4516655fac8"
+ value: "Sanjeeva P. Kalva, MD, FSIR, FCIRSE"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Mycotic Aneurysm"
+ slug: "mycotic-aneurysm"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "8f0b7338-cf37-4f0c-ad92-25741a515b79"
+imageCount: 18
+lastUpdated: "11/20/24"
+pageDescription: "Mycotic Aneurysm"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Mycotic Aneurysm"
+pageTitle: "Mycotic Aneurysm | STATdx"
+enhancedTitle: "Mycotic Aneurysm"
+type: "DX"
+references: true
+ddx: true
+cases: 1
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Mycotic Aneurysm"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Aneurysm arising from infection of arterial wall, usually bacterial
+- ## Imaging
+
+
+ - Rapidly growing focal saccular aneurysm arising eccentrically from aortic wall
+ - Periaortic soft tissue stranding, edema, and fluid
+ - Adjacent vertebral body or psoas abnormalities due to spread of infection
+ - Increased uptake of labeled leukocytes at site of aneurysm
+- ## Top Differential Diagnoses
+
+
+ - Atherosclerotic aneurysm
+ - Inflammatory aneurysm
+ - Contained rupture
+ - Aortoenteric fistula
+- ## Pathology
+
+
+ - Bacterial aortitis most commonly caused by *Salmonella* or *Staphylococcus aureus*
+ - Primary mycotic aneurysm arises from distant, unknown, or remote source of infection
+ - Secondary mycotic aneurysm arises from specific source of infection
+- ## Clinical Issues
+
+
+ - Fever, signs of sepsis
+ - Positive blood cultures in most cases
+ - Surgical resection/grafting following antibiotic therapy
+- ## Diagnostic Checklist
+
+
+ - Contrast-enhanced CTA or MRA with delayed images for evaluation
+ - Labeled leukocyte scan if indeterminate CTA and MRA
+
+# TERMINOLOGY
+
+- ## Synonyms
+
+
+ - Infectious aneurysm (more appropriate term)
+- ## Definitions
+
+
+ - Aneurysm arising from infection of arterial wall, usually bacterial
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Rapidly growing saccular aneurysm arising eccentrically from aortic wall
+ - ### Location
+
+
+ - Anywhere in aorta or other vessels
+ - Tends to occur at major branch points of aorta
+ - ### Size
+
+
+ - Variable
+ - ### Morphology
+
+
+ - Usually saccular with focal involvement of artery
+ - Periaortic inflammation, abscess, mass
+ - Periaortic gas
+ - Adjacent vertebral body abnormalities due to spread of infection
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - May reveal increased size of aorta
+ - Lytic or sclerotic areas in adjacent bone
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - Periaortic soft tissue stranding, edema, and fluid are frequent
+ - Periaortic gas
+ - Adjacent vertebral body or psoas abnormalities due to spread of infection
+ - Periaortic, high-attenuation fluid if ruptured
+ - Bacterial aortitis is rarely calcified
+ - Syphilitic aortitis shows curvilinear calcifications
+ - ### CECT
+
+
+ - ≥ 1 saccular aneurysm(s) arising from aortic wall, usually focal
+ - Lobular contours of aneurysm
+ - Enhancement of periaortic soft tissue
+ - Rim enhancement in case of abscess
+ - ### CTA
+
+
+ - Saccular, eccentric aneurysms of variable size
+ - Enhancing periaortic soft tissue or abscess
+- ## MR Findings
+
+
+ - ### T1WI
+
+
+ - Periaortic low signal intensity in nonenhanced MR
+ - Aortic and periaortic enhancement following gadolinium, especially evident on fat-suppressed images
+ - Rim enhancement in case of abscess
+ - Adjacent bone abnormality if contiguous infection
+ - ### T2WI
+
+
+ - Periaortic high signal intensity on fat-suppressed T2WI
+ - Contrast-enhanced MRA
+ - ≥ 1 saccular aneurysm(s) arising from aortic wall
+ - Effacement of wall with possible leakage at rupture site
+ - In addition to MRA, delayed source images need to be analyzed to identify areas of enhancement
+- ## Ultrasonographic Findings
+
+
+ - ### Grayscale ultrasound
+
+
+ - Useful in children or if superficial arteries are involved
+ - Focal, eccentric pseudoaneurysm
+ - Perivascular soft tissue or abscess
+ - ### Color Doppler
+
+
+ - Flow within aneurysm with typical yin-yang configuration
+- ## Echocardiographic Findings
+
+
+ - ### Echocardiogram
+
+
+ - Used to rule out endocarditis as potential source of septic emboli
+- ## Angiographic Findings
+
+
+ - Conventional
+ - Focal, saccular aneurysm
+ - Irregularity of luminal surface
+- ## Nuclear Medicine Findings
+
+
+ - ### Labeled leukocyte scintigraphy
+
+
+ - Increased uptake at site of aneurysm
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - Contrast-enhanced CT/CTA
+ - Labeled leukocyte scintigraphy
+ - ### Protocol advice
+
+
+ - Obtain delayed images during contrast-enhanced CTA or MRA
+ - Review adjacent bones
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Atherosclerotic Aneurysm
+
+
+ - Slow growing
+ - More often fusiform
+ - Often calcified
+ - No enhancement of aortic wall
+- ## Inflammatory Aneurysm
+
+
+ - Distal aorta and iliac involvement
+ - Thick rind of soft tissue around aorta
+ - Uniform, rim-like aortic wall enhancement on contrast CT/MR
+ - Fusiform aneurysm
+ - Retroperitoneal fibrosis
+- ## Contained Rupture
+
+
+ - Focal disruption or gap in aortic wall
+ - High attenuation in wall or in periphery of aneurysm
+ - Lack of enhancement
+- ## Aortoenteric Fistula
+
+
+ - Most involve duodenum
+ - Periaortic soft tissue with periaortic gas
+ - Active contrast material extravasation or pseudoaneurysm
+ - Presents as gastrointestinal bleed
+- ## Surgical material
+
+
+ - History of prior surgery
+ - Hyperdense on NECT
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Bacterial aortitis most commonly caused by *Salmonella* or *Staphylococcus aureus*
+ - Syphilitic aortitis involves ascending aorta but spares aortic sinus: Ascending aorta most common location
+ - Routes of infection
+ - Most often caused by seeding of existing lesion (atheroma or aneurysm) via vasa vasorum
+ - Direct extension from infection in vessel wall, i.e., bacterial endocarditis
+ - Invasion of aortic wall by extravascular contiguous infection, such as spinal infection or intraabdominal abscess
+ - Lymphatic spread
+ - *Burkholderia pseudomallei* (causing melioidosis), endemic in Southeast Asia and Northern Australia, is increasingly recognized as agent causing aortitis and mycotic aneurysms
+ - ### Associated abnormalities
+
+
+ - Endocarditis
+ - Spinal or retroperitoneal infection
+ - Intraabdominal infection
+- ## Staging, Grading, & Classification
+
+
+ - Classification system
+ - Primary mycotic aneurysm arises from distant, unknown, or remote source of infection
+ - Secondary mycotic aneurysm arises from specific source of infection
+ - Bacterial endocarditis (intravascular spread)
+ - Tuberculosis (contiguous spread)
+- ## Gross Pathologic & Surgical Features
+
+
+ - Bacterial aneurysm
+ - Noncalcified saccular aneurysm
+ - Thinning of aortic wall with periaortic inflammatory changes
+ - Syphilitic aneurysm
+ - Calcified lesion
+ - Tree bark appearance when atheroma develops in infected areas
+- ## Microscopic Features
+
+
+ - Loss of intima and destruction of internal elastic lamina
+ - Varying degrees of destruction of media
+ - Bacteria present on histology
+ - Common bacteria: *Pseudomonas*, *Clostridium*, *Salmonella*, *Streptococcus*, *Aspergillus*
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Fever, signs of sepsis
+ - Symptoms vary greatly
+ - Nonspecific findings
+ - Low-grade fever
+ - Localized pain
+ - Positive blood cultures
+ - Blood cultures are negative in 25% of cases
+- ## Demographics
+
+
+ - ### Epidemiology
+
+
+ - 0.7-2.6% of all aortic aneurysms
+ - Increased risk in
+ - Intravenous drug abusers
+ - Patients with history of bacterial endocarditis
+ - Occurs in 2% of patients with infective endocarditis
+ - Most common location is intracranial
+ - Rupture more easily and associated with poor prognosis
+ - Immunocompromised patients
+ - Patients with vascular prostheses (valves, grafts)
+- ## Natural History & Prognosis
+
+
+ - Nearly always fatal if untreated
+ - Acute rupture/hemorrhage seen in 75%
+ - Mortality rate estimated at 67%
+- ## Treatment
+
+
+ - Surgical resection/grafting following antibiotic therapy
+ - May need extraanatomic bypass grafting
+ - Endovascular repair in some cases
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - Contrast-enhanced CTA or MRA with delayed images for evaluation
+ - Labeled leukocyte scan if CTA and MRA are indeterminate
+- ## Image Interpretation Pearls
+
+
+ - Focal, eccentric aneurysm of aorta
+ - Enhancing periaortic soft tissue
+ - Rim enhancement of periaortic abscess
+- ## Reporting Tips
+
+
+ - Include location, size, and involvement of branch vessels
+ - Check for and report extent of contiguous infection
+
+ a1eb2f8e-cf67-4cad-84d1-41c6f82b7d53
+
+## References
+
+# Selected References
+
+1. [Calderón-Parra J et al: Epidemiology and risk factors of mycotic aneurysm in patients with infective endocarditis and the impact of its rupture in outcomes. Analysis of a national prospective cohort. Open Forum Infect Dis. 11(3):ofae121, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38500574%5Bpmid%5D)
+1. [Wyss TR et al: Infective native aortic aneurysm: a Delphi consensus document on treatment, follow up, and definition of cure. Eur J Vasc Endovasc Surg. 67(4):654-61, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38097164%5Bpmid%5D)
+1. [Wu H et al: Mycotic aneurysm secondary to melioidosis in China: a series of eight cases and a review of literature. PLoS Negl Trop Dis. 14(8):e0008525, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32785225%5Bpmid%5D)
+1. [Haidar GM et al: "In situ" endografting in the treatment of arterial and graft infections. J Vasc Surg. 65(6):1824-9, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28359717%5Bpmid%5D)
+1. [Sörelius K et al: Endovascular treatment of mycotic aortic aneurysms: a paradigm shift. J Cardiovasc Surg (Torino). 58(6):870-4, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28627863%5Bpmid%5D)
+1. [Deipolyi AR et al: Imaging findings, diagnosis, and clinical outcomes in patients with mycotic aneurysms: single center experience. Clin Imaging. 40(3):512-6, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27133696%5Bpmid%5D)
+1. [Murphy DJ et al: Cross-sectional imaging of aortic infections. Insights Imaging. 7(6):801-18, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27761883%5Bpmid%5D)
+1. [Uchida N et al: In situ replacement for mycotic aneurysms on the thoracic and abdominal aorta using rifampicin-bonded grafting and omental pedicle grafting. Ann Thorac Surg. 93(2):438-42, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22054654%5Bpmid%5D)
+1. [Iida H et al: Bacteremia causes mycotic aneurysm of the aortic arch in 110 days. Ann Thorac Surg. 83(5):1874-6, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17462421%5Bpmid%5D)
+1. [Taylor CF et al: Treatment options for primary infected aorta. Ann Vasc Surg. 21(2):225-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17349369%5Bpmid%5D)
+1. [Froeschl M et al: Ruptured mycotic pseudoaneurysm of the thoracic aorta. Cardiovasc Pathol. 15(2):116-8, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16533702%5Bpmid%5D)
+1. [Kerzmann A et al: Infected abdominal aortic aneurysm treated by in situ replacement with cryopreserved arterial homograft. Acta Chir Belg. 106(4):447-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17017707%5Bpmid%5D)
+1. [Lee KH et al: Stent-graft treatment of infected aortic and arterial aneurysms. J Endovasc Ther. 13(3):338-45, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16784321%5Bpmid%5D)
+1. [Palanichamy N et al: Mycotic pseudo-aneurysm of the ascending thoracic aorta after cardiac transplantation. J Heart Lung Transplant. 25(6):730-3, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16730580%5Bpmid%5D)
+1. [Ting AC et al: Endovascular stent graft repair for infected thoracic aortic pseudoaneurysms--a durable option? J Vasc Surg. 44(4):701-5, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16930927%5Bpmid%5D)
+1. [Gonzalez-Fajardo JA et al: Endovascular repair in the presence of aortic infection. Ann Vasc Surg. 19(1):94-8, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15714375%5Bpmid%5D)
+1. [Ting AC et al: Surgical treatment of infected aneurysms and pseudoaneurysms of the thoracic and abdominal aorta. Am J Surg. 189(2):150-4, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15720981%5Bpmid%5D)
+1. [Malouf JF et al: Mycotic aneurysms of the thoracic aorta: a diagnostic challenge. Am J Med. 115(6):489-96, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14563506%5Bpmid%5D)
+1. [Cina CS et al: Ruptured mycotic thoracoabdominal aortic aneurysms: a report of three cases and a systematic review. J Vasc Surg. 33(4):861-7, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11296343%5Bpmid%5D)
+1. [Locati P et al: Salmonella mycotic aneurysms: traditional and "alternative" surgical repair with arterial homograft. Minerva Cardioangiol. 47(1-2):31-7, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10356939%5Bpmid%5D)
+1. [Long R et al: Tuberculous mycotic aneurysm of the aorta: review of published medical and surgical experience. Chest. 115(2):522-31, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10027455%5Bpmid%5D)
+1. [Fichelle JM et al: Infected infrarenal aortic aneurysms: when is in situ reconstruction safe? J Vasc Surg. 17(4):635-45, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8464080%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Dilated Aorta
+DDX:9daee273-f1e9-4cf9-a979-8990a9b82e40
+
+### Dilated Aorta
+DDX:bb8315f3-0893-4f17-aaf6-343fd5419b8e
+
+## Cases
+
+- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'cf6a6efe-995c-4a18-9cb2-5f3395aae678', 'description': "Variant case of a rare mycotic aneurysm presumably from extension from the adjacent thoracic osteomyelitis. The patient's hemoptysis was from the additional complication of an aortobronchial fistula. Patient was emergently treated with a covered stent within the descending aorta.\n\nCTA study of the thorax (#1-8) shows typical appearance of a midthoracic disc space infection with collapse of the disc space and endplate destruction (arrow, #1). Midline sagittal image (#1) also shows adjacent soft tissue mass within the posterior mediastinum (open arrows, #1). The contrast-enhanced images show a large, irregular, enhancing lesion consistent with pseudoaneurysm (open arrows, #2-5) within the posterior mediastinum that displaces the aorta anteriorly (curved arrows, #3-5). The vertebral body destruction from the osteomyelitis (arrows, #3-5) and the extension into the prevertebral soft tissues and mediastinum is seen on the axial views. Coronal images (#6-8) show the pseudoaneurysm (arrows) and the surrounding inflammatory mass as well as the disc space centered bony destruction (open arrow, #8).\n\nOblique aortogram shows contrast extravasating into the pseudoaneurysm with a broad neck (arrows, #9,10).", 'history': 'Presented with massive hemoptysis; history of back pain.', 'imagePoolId': 'd5da4f47-3ed7-4e07-9c88-8dcf8bb6ae26', 'name': 'Aortic vertebral fistula and aortobronchial fistula', 'teachingPoint': None, 'demographics': '59 Years old male'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial CECT of the abdominal aorta shows periaortic, low-density soft tissue with rim enhancement of the aortic wall, which is consistent with an infected aortic wall and periaortic abscess.*
+
+
+*Axial CECT of the abdominal aorta shows periaortic, low-density soft tissue with rim enhancement of the aortic wall, which is consistent with an infected aortic wall and periaortic abscess.*
+
+
+*Axial CECT of the aorta in the same patient shows an area of focal, small luminal outpouching (pseudoaneurysm) of the left lateral wall of the aorta with associated periaortic soft tissue swelling , consistent with a mycotic aneurysm.*
+
+
+*Oblique CTA reconstruction in the same patient shows 2 focal contrast outpouchings consistent with mycotic pseudoaneurysms affecting the lateral wall of the abdominal aorta.*
+
+
+*Coronal CTA of the aortoiliac arteries in the same patient following infrarenal aortic resection shows that the lower extremities are now perfused via a right axillary-femoral artery bypass graft and cross-femoral bypass graft . Note the absence of resected infrarenal aorta.*
+
+
+*Axial CTA of the abdominal aorta shows a focal, eccentric pseudoaneurysm affecting the juxtarenal abdominal aorta . Note the minimal periaortic soft tissue.*
+
+
+*Oblique CTA of the abdominal aorta in the same patient shows a focal, eccentric pseudoaneurysm . There is no significant soft tissue adjacent to the pseudoaneurysm. This patient had bacteremia and spinal infection (not shown). Surgical resection of the aorta confirmed the mycotic nature of the pseudoaneurysm.*
+
+
+*Axial CTA of the thoracic aorta shows a focal, eccentric pseudoaneurysm arising from the anterior wall of the ascending aorta with associated periaortic soft tissue .*
+
+
+*Oblique CTA of the thoracic aorta in the same patient shows a focal, eccentric pseudoaneurysm along the anterior wall of the ascending aorta with associated low-density soft tissue . These features are consistent with a mycotic aneurysm.*
+
+
+*Axial CTA of the abdomen shows a pseudoaneurysm arising from a branch of the superior mesenteric artery. Note the perianeurysmal soft tissue .*
+
+
+*Coronal CTA in the same patient confirms the pseudoaneurysm arising from a branch of the superior mesenteric artery. This was secondary to a septic embolus from valvular vegetations in a 30-year-old man with endocarditis secondary to intravenous drug abuse.*
+
+
+### Additional Images
+
+
+*Oblique CTA shows a saccular aortic aneurysm with peripheral mural thrombus . Mycotic aneurysms are usually saccular and involve a focal arterial segment. Infection weakens the arterial wall and allows for the aneurysm formation.*
+
+
+*Axial CECT shows a saccular aortic aneurysm with mural thrombus.*
+
+
+*Axial CECT shows the saccular aneurysm with mural thrombus arising from the abdominal aorta . Mycotic aneurysms typically involve a diseased segment of the arterial wall.*
+
+
+*Oblique MRA shows multiple small pseudoaneurysms following the ascending aortic aneurysm repair. Also note the enhancing soft tissue surrounding the aneurysms.*
+
+
+*Axial NECT shows a saccular outpouching from the abdominal aorta . This eccentric saccular appearance is typical of a mycotic aneurysm.*
+
+
+*Axial CECT shows a pseudoaneurysm and periaortic soft tissue following surgical repair of an ascending aortic aneurysm.*
+
+
+*Axial CECT shows a saccular aortic aneurysm with mural thrombus and periaortic soft tissue .*
+
+
+*Coronal CTA shows a saccular aortic aneurysm arising from the lateral wall of the abdominal aorta. Mycotic aneurysms account for ≤ 2.6% of all aortic aneurysms. There is an increased risk for this type of aneurysm in intravenous drug abusers, immunocompromised patients, and in cases of bacterial endocarditis.*
+
diff --git a/docs_md/articles/narrowed-aorta_763503a4-a7b8-4aff-8846-3dfbe312125c.md b/docs_md/articles/narrowed-aorta_763503a4-a7b8-4aff-8846-3dfbe312125c.md
new file mode 100644
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--- /dev/null
+++ b/docs_md/articles/narrowed-aorta_763503a4-a7b8-4aff-8846-3dfbe312125c.md
@@ -0,0 +1,128 @@
+---
+title: "Narrowed Aorta"
+docid: "763503a4-a7b8-4aff-8846-3dfbe312125c"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Narrowed Aorta"
+ slug: "narrowed-aorta"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "d3c26ea4-8f08-4b3c-8e42-c5d40e18e83c"
+imageCount: 9
+lastUpdated: "03/17/22"
+pageDescription: "Narrowed Aorta"
+pageKeywords: "Cardiac, Differential Diagnosis, Narrowed Aorta"
+pageTitle: "Narrowed Aorta | STATdx"
+enhancedTitle: "Narrowed Aorta"
+type: "DDX"
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Narrowed Aorta"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Focal outer diameter narrowed in coarctation or vasculitis
+ - Normative data should be consulted to exclude common pitfall of misinterpreting normal aortic dimension for small body as abnormal
+ - For > 45 years, aorta considered abnormally small if diameter at level of main pulmonary artery < 24 mm for ascending and < 18 mm for descending aorta
+ - Larger diameters may still be abnormal if older age, larger body surface area (BSA), or male patient
+ - Luminal diameter narrowing in atherosclerosis, aortic thrombus, and sarcoma
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Coarctation of Aorta**
+ - Focal narrowing occurs below ductus arterious in adults, at ductus arterious with arch hypoplasia in neonates
+ - Dilated collaterals (intercostal, internal thoracic) indicate hemodynamically significant coarctation
+ - Treated coarctation patients can have restenosis
+ - Associated with bicuspid aortic valve, Turner syndrome, Marfan syndrome
+ - **Pseudo-coarctation**
+ - Redundant aorta with narrowing distal to left subclavian origin without hemodynamic effect
+ - No rib notching, cardiomegaly, or collateral vessels
+ - Dilated brachiocephalic artery and high arch often present
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Corral Reef Aorta**
+ - "Rock hard" calcific atherosclerotic plaque protruding into lumen of abdominal aorta
+ - Bizarre shape of protrusions resemble corals
+ - **Leriche Syndrome**
+ - Aortoiliac occlusive disease due to severe atherosclerosis of distal abdominal aorta and ileo-femoral arteries
+ - Presenting symptom triad in chronic variant: Claudication, absent femoral pulses, erectile dysfunction
+ - **Large Vessel Vasculitis**
+ - Variable segment length narrowing
+ - Branch vessel narrowing is common
+ - Periaortic thickening and enhancement
+ - Periaortic FDG uptake implies active disease
+ - Patient < 40 years of age implies Takayasu, patient > 40 years of age implies giant cell arteritis
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Extrinsic Aortic Compression**
+ - Retroperitoneal fibrosis, neurofibromatosis, sarcomas, other neoplasms
+ - **Aortic Sarcoma**
+ - Rare mesenchymal neoplasms arising from aortic tissue and may protrude into and narrow aortic lumen
+ - Angiosarcoma, leiomyosarcoma, fibrosarcoma, hemangioendothelioma, and myxoid sarcomas have been reported
+
+
+## Images
+
+
+### Selected Images
+
+
+**Coarctation of Aorta**
+*PA radiograph shows subtle areas of rib notching . Note the rapid narrowing of the proximal descending thoracic aorta , which then returns to normal size more distally .*
+
+
+**Coarctation of Aorta**
+*PA radiograph shows subtle areas of rib notching . Note the rapid narrowing of the proximal descending thoracic aorta , which then returns to normal size more distally .*
+
+
+**Coarctation of Aorta**
+*Sagittal multiplanar CT reformat in the same patient shows focal narrowing of the aorta distal to the ductus arteriosus . Prominent collaterals were present (not shown).*
+
+
+**Pseudo-coarctation**
+*Sagittal oblique NECT shows focal mild narrowing of the aorta in a patient with pseudo-coarctation. The aorta appears redundant. No collateral vessels were present.*
+
+
+**Extrinsic Aortic Compression**
+*Axial CECT shows extrinsic compression of the aorta by retroperitoneal soft tissue in a patient with retroperitoneal fibrosis.*
+
+
+**Large Vessel Vasculitis**
+*Coronal oblique contrast-enhanced MRA shows a diffusely narrowed aorta occlusion of the superior mesenteric artery . Note reconstituted SMA .*
+
+
+**Large Vessel Vasculitis**
+*Sagittal oblique MIP MRA in a patient with Takayasu arteritis shows tapered narrowing of the mid-descending thoracic aorta . Celiac artery origin stenosis is also present .*
+
+
+**Large Vessel Vasculitis**
+*Sagittal oblique MIP CECT shows tapered narrowing of the mid-descending aorta with wall thickening in a patient with Takayasu arteritis. Note the absence of atherosclerotic disease.*
+
+
+**Large Vessel Vasculitis**
+*Axial CECT shows a narrowed aorta with circumferential wall thickening . Branch vessel stenoses were also present. This patient was > 40 years of age and was diagnosed with giant cell arteritis.*
+
+
+### Additional Images
+
+
+**Large Vessel Vasculitis**
+*Coronal CECT shows tapered narrowing of the mid-descending thoracic aorta in a patient with Takayasu arteritis.*
+
diff --git a/docs_md/articles/penetrating-atherosclerotic-ulcer_63f6cba2-2200-456a-8d03-ac3111e420c8.md b/docs_md/articles/penetrating-atherosclerotic-ulcer_63f6cba2-2200-456a-8d03-ac3111e420c8.md
new file mode 100644
index 0000000..5c461b5
--- /dev/null
+++ b/docs_md/articles/penetrating-atherosclerotic-ulcer_63f6cba2-2200-456a-8d03-ac3111e420c8.md
@@ -0,0 +1,412 @@
+---
+title: "Penetrating Atherosclerotic Ulcer"
+docid: "63f6cba2-2200-456a-8d03-ac3111e420c8"
+authors:
+ - key: "9ad9af12-61a1-44d6-af52-0ee1f38eb298"
+ value: "Davis Vigneault, MD, DPhil"
+ - key: "5de0df07-7b3e-4678-8767-1519e1153f29"
+ value: "Dominik Fleischmann, MD"
+ - key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
+ value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Penetrating Atherosclerotic Ulcer"
+ slug: "penetrating-atherosclerotic-ulcer"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "412ad523-b2df-4d8e-a1cb-537301c9c463"
+imageCount: 28
+lastUpdated: "01/24/25"
+pageDescription: "Penetrating Atherosclerotic Ulcer"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Penetrating Atherosclerotic Ulcer"
+pageTitle: "Penetrating Atherosclerotic Ulcer | STATdx"
+enhancedTitle: "Penetrating Atherosclerotic Ulcer"
+type: "DX"
+references: true
+ddx: true
+anatomy:
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+cases: 1
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Penetrating Atherosclerotic Ulcer"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Ulceration of atherosclerotic plaque that penetrates internal elastic lamina into media with variable amount of intramural hematoma (IMH)
+- ## Imaging
+
+
+ - NECT: Abnormal aortic contour if penetrating atherosclerotic ulcer (PAU) has penetrated beyond adventitia; possible IMH if acute
+ - CTA: Contrast outpouching extending beyond expected depth of aortic intima ± IMH
+ - MR: Similar sensitivity and findings to CTA, but less practical in emergent evaluation of acute aortic syndrome
+- ## Top Differential Diagnoses
+
+
+ - Ulcerated atherosclerotic plaque (nonpenetrating ulcer)
+ - Intimomedial disruptions in IMH (focal intimal disruption, intramural blood pool)
+ - Chronic healed PAU
+ - Mycotic pseudoaneurysm
+- ## Pathology
+
+
+ - Stanford classification for aortic dissection
+ - Type A: Ascending ± arch/descending aorta
+ - Type B: Ascending **not** involved (only descending ± arch)
+ - Variable IMH from erosion of vasa vasorum
+ - Classification of AAS (Svensson)
+ - Class I: Classical dissection
+ - Class II: Intramural hematoma or hemorrhage
+ - Class III: Subtle dissection without hematoma
+ - Class IV: Penetrating aortic ulcer
+ - Class V: Iatrogenic or traumatic dissection
+- ## Clinical Issues
+
+
+ - Acute: Chest/back pain in thoracic aorta, abdominal/back/flank pain in abdominal aorta
+ - Chronic: Common incidental finding, asymptomatic, never associated with IMH
+ - Older adults (typically 7th decade or later), M > F
+ - Risk factors: Hypertension, tobacco use, coronary artery disease, chronic obstructive pulmonary disease, and renal insufficiency
+ - Concomitant aortic aneurysm is common
+ - Acute PAU (symptomatic ± IMH) are more likely to progress to perforation or aortic rupture
+ - Chronic healed PAU (asymptomatic without IMH) are unlikely to progress
+ - Consider treatment if symptomatic, complicated, or large
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Penetrating atherosclerotic ulcer (PAU)
+- ## Definitions
+
+
+ - Ulceration of atherosclerotic plaque penetrating through internal elastic lamina into media or beyond
+ - Acute aortic syndrome (AAS): Clinical syndrome describing acute aortic pain syndrome (sharp, tearing, very abrupt chest or back pain) in contradistinction to acute coronary syndrome (more dull, pressure-like, gradual onset)
+ - Most common pathologies resulting in symptoms of AAS are classic dissection, intramural hematoma (IMH), and PAU, but also include limited tears and rupturing thoracic aortic aneurysms
+ - Traumatic aortic injuries and iatrogenic dissections (e.g., as complication of percutaneous intervention or associated with surgery) are usually **not** considered AAS (since they do not "present" with spontaneous acute symptoms), although they are acute pathologic entities
+ - AAS is clinical pain syndrome, not specific pathology
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Contrast extending beyond expected depth of intima in setting of atherosclerosis
+ - ### Location
+
+
+ - Most commonly involves mid- to distal descending thoracic aorta
+ - Less commonly involves aortic arch and ascending or abdominal aorta
+ - Patients with acute PAU commonly have chronic atherosclerotic ulcers and aneurysms
+ - Sometimes > 1 PAU can be seen
+- ## Radiographic Findings
+
+
+ - Chest radiography is insensitive and often normal
+ - Pleural effusion is common when acute
+ - May be helpful for identifying other causes of chest pain
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - ± focally abnormal aortic contour if PAU extends beyond adventitia
+ - May be obscured by adjacent IMH
+ - ± adjacent concentric or crescentic hyperattenuating intramural hematoma (IMH) (if acute)
+ - Associated calcified atherosclerotic plaques
+ - ### CTA
+
+
+ - Intraluminal contrast extending beyond expected depth of intima; ± focal adjacent IMH if acute
+ - Often better appreciated on NECT
+ - Adjacent hemorrhage (i.e., contained rupture or pseudoaneurysm); may progress to aortic rupture
+- ## MR Findings
+
+
+ - ### MRA
+
+
+ - Similar findings and sensitivity to CTA, but is less cost-effective and less practical in emergent acute aortic syndrome (AAS) evaluation
+- ## Angiographic Findings
+
+
+ - Conventional
+ - Outpouching (ulcer) along descending thoracic aorta
+ - Luminal irregularity from diffuse atherosclerotic plaque
+- ## Imaging Recommendations
+
+
+ - ### Protocol advice
+
+
+ - Obtain NECT before CTA to help identify IMH
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Ulcerated Atherosclerotic Plaque
+
+
+ - Ulceration confined to aortic intima; does not extend beyond expected depth of intima
+ - These non-PAUs are extremely common and present in almost all individuals with advanced atherosclerotic disease
+ - Ulcerated plaques (a.k.a. ruptured plaques) in side branches of aorta cause most of acute cardiovascular events
+ - Myocardial infarct if in coronary arteries; stroke if in cerebral vasculature
+- ## Intimomedial Disruptions in Intramural Hematoma
+
+
+ - Focal intimal disruption: > 0.3-cm orifice, representing isolated entry tear, more likely to progress
+ - Additional terms used in literature include ulcer-like projection and isolated primary intimal tear
+ - Intramural blood pool (IBP): < 0.3-cm orifice, representing avulsed branch vessel (therefore descending aorta only), more likely to spontaneously resolve
+ - Additional terms used in literature include natural fenestrations, branch artery pseudoaneurysms, and focal puddles (corresponding to IBP)
+ - Does not communicate with aortic lumen
+- ## Chronic Healed Penetrating Atherosclerotic Ulcer
+
+
+ - Asymptomatic and no IMH = unlikely to progress
+- [Traumatic Aortic Injury](/document/traumatic-aortic-injury/e32745ac-4438-41cc-a44a-7106fbbfc657)
+ - History of trauma/additional traumatic findings; not associated with atherosclerosis
+ - Aortic isthmus (most common), diaphragmatic hiatus, or aortic root
+- [Mycotic Pseudoaneurysm](/document/mycotic-aneurysm/20616d0a-7e0b-48d9-9be9-1af29e3dd6da)
+ - Clinical evidence of infection; not associated with atherosclerosis; can be morphologically similar to PAU
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Manifestation of advanced atherosclerosis
+ - Only very small fraction of atherosclerotic ulcers penetrate deeper than into thickened intima, into media of aorta or beyond
+ - ### Associated abnormalities
+
+
+ - Coexistent aortic aneurysms, coexisting nonpenetrating ulcers, or chronic (healed) PAUs
+ - Location
+ - Descending thoracic aorta is most commonly affected
+- ## Staging, Grading, & Classification
+
+
+ - Stanford classification (for aortic dissection)
+ - Type A: Ascending aorta
+ - Type B: Aortic arch &/or descending aorta
+- ## Gross Pathologic & Surgical Features
+
+
+ - Variable presence/degree of IMH
+- ## Microscopic Features
+
+
+ - Disruption of internal elastic lamina and extension into media
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Acute-onset sharp chest/back pain in thoracic aorta, abdominal/back/flank pain in abdominal aorta
+ - Chronic: Often seen on imaging in patients with atherosclerosis; acute PAUs can heal, reendothelialize, and become chronic
+ - In chronic phase, they can grow slowly over time and become saccular, eccentric aneurysms
+ - Embolization of atheroma (uncommon): Atherosclerotic plaque ulceration can lead to atheroembolism into downstream vessels
+ - Classic manifestation is "blue toe syndrome'" due to microembolic occlusion of small peripheral arteries
+ - Atheroembolism is related to superficial plaque rupture, not to deep penetration, but these can coincide
+- ## Demographics
+
+
+ - ### Epidemiology
+
+
+ - M > F; PAU represents 2-7% of all AASs
+ - Classic atherosclerosis risk factors, established atherosclerotic diseases
+ - Associated concomitant diseases: Hypertension, tobacco use, coronary artery disease, cerebrovascular disease, peripheral artery disease, chronic obstructive pulmonary disease, and renal insufficiency; history of myocardial infarction, stroke, aortic aneurysms, peripheral artery disease
+ - Concomitant aortic aneurysm is common
+ - Age: Older adults (typically 7th decade or later)
+- ## Natural History & Prognosis
+
+
+ - Evolution of PAU
+ - Acute PAU (symptomatic ± IMH) can → aortic rupture
+ - If associated with IMH, CT attenuation of mural thrombus decreases and becomes isodense to blood pool within 7-10 days on NECT
+ - Chronic, healed PAUs are usually asymptomatic; they can remodel over time and grow gradually over years
+ - They may become saccular aneurysms
+ - High-risk features: Diameter ≥ 13-20 mm, depth ≥ 10 mm, significant growth, associated saccular aneurysm, or increasing pleural effusion
+- ## Treatment
+
+
+ - Medical management if uncomplicated
+ - Open or endovascular surgical intervention if complicated
+ - Rupture (impending), malperfusion, uncontrollable pain/hypertension
+ - Endovascular therapy is preferred over open surgical repair in anatomically suitable candidates
+
+ b8b3e292-9b6b-4cb3-9d4d-f431e6d3c8c9
+
+## References
+
+# Selected References
+
+1. [Isselbacher EM et al: 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology joint committee on clinical practice guidelines. Circulation. 146(24):e334-482, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36322642%5Bpmid%5D)
+1. [DeCarlo C et al: Prognostication of asymptomatic penetrating aortic ulcers: a modern approach. Circulation. 144(14):1091-101, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34376058%5Bpmid%5D)
+1. [Ishizu K et al: Focal intimal disruption size at multidetector CT and disease progression in type B aortic intramural hematoma. Radiology. 301(2):311-9, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34374587%5Bpmid%5D)
+1. [Raptis CA et al: Intramural hematoma and focal intimal disruption: the importance of communication. Radiology. 301(2):320-1, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34374599%5Bpmid%5D)
+1. [Vilacosta I et al: Acute aortic syndrome revisited: JACC state-of-the-art review. J Am Coll Cardiol. 78(21):2106-25, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34794692%5Bpmid%5D)
+1. [Evangelista A et al: Penetrating atherosclerotic ulcer. Curr Opin Cardiol. 35(6):620-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32925190%5Bpmid%5D)
+1. [Bossone E et al: Acute aortic syndromes: diagnosis and management, an update. Eur Heart J. 39(9):739-49d, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29106452%5Bpmid%5D)
+1. [El Hassani I et al: Penetrating atherosclerosis aortic ulcer: a re-appraisal. Acta Chir Belg. 117(1):1-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27593515%5Bpmid%5D)
+1. [Gutschow SE et al: Emerging Concepts In Intramural Hematoma Imaging. Radiographics. 36(3):660-74, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27163587%5Bpmid%5D)
+1. [Patatas K et al: Penetrating atherosclerotic ulcer of the aorta: a continuing debate. Clin Radiol. 68(8):753-9, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23541075%5Bpmid%5D)
+1. [Akin I et al: Penetrating aortic ulcer, intramural hematoma, acute aortic syndrome: when to do what. J Cardiovasc Surg (Torino). 53(1 Suppl 1):83-90, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22433727%5Bpmid%5D)
+1. [Bischoff MS et al: Penetrating aortic ulcer: defining risks and therapeutic strategies. Herz. 36(6):498-504, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21887528%5Bpmid%5D)
+1. [Vilacosta I et al: Acute aortic syndrome: a new look at an old conundrum. Postgrad Med J. 86(1011):52-61, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20065341%5Bpmid%5D)
+1. [François CJ et al: MRI of the thoracic aorta. Cardiol Clin. 25(1):171-84, vii, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17478245%5Bpmid%5D)
+1. [Hayashi H et al: Penetrating atherosclerotic ulcer of the aorta: imaging features and disease concept. Radiographics. 20(4):995-1005, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10903689%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Aortic Intramural Abnormality
+DDX:75d7b37f-bc37-493b-8961-8b2a9001fb94
+
+### Dilated Aorta
+DDX:9daee273-f1e9-4cf9-a979-8990a9b82e40
+
+## Anatomy
+
+### Aortic Arch and Great Vessels
+Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
+
+### Vessels, Lymphatic System, and Nerves, Abdominal
+Gastrointestinal/ANATOMY:0c38fd49-88e7-4272-960f-b17a194ce0cc
+
+### Aorta and Inferior Vena Cava
+Ultrasound/ANATOMY:eed39e4d-478b-45d3-9406-1eace6e9eef1
+
+### Abdominal Aorta and Visceral Vasculature Anatomy
+Vasculature/ANATOMY:341c0af5-bfe8-4755-a401-39b7b9f2d9b9
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '2cd036e9-d3d3-4b44-93f1-c0f8bf83ac5e', 'value': None}, {'key': 'd69a0534-8989-4b83-b19d-c3b640f22e53', 'value': 'Megan R. Saettele, MD'}, {'key': '2e78a4ff-418e-46f9-93e4-c6d95d960e35', 'value': 'Brandt C. Wible, MD'}], 'caseVersionId': '9fb96b0f-ca0f-4791-906a-b5842feee3d3', 'description': 'Unenhanced CT imaging of the abdomen was performed for clinical suspicions of a functional adrenal adenoma (#1-2). No such abnormality was found. However, a small left posterior outpouching from the infrarenal abnormal aorta was incidentally seen (white arrows, #1-2), this finding consistent with a small aortic ulcer.\n\nApproximately 1.5 years later, the patient began complaining of claudication. An abdominal aortic angiogram was subsequently performed (#3), and a frontal projection from this study demonstrates several focal outpouchings from the distal abdominal aorta (black arrows, #3). A coned-in sagittal image shows a large posterior ulceration from the abdominal aorta (black arrow, #4).\n\nComment: Aortic ulcerations (also known as penetrating atherosclerotic aortic ulcers) are associated with a relatively high rate of aortic rupture, particularly those greater than 2 cm in diameter or 1 cm in depth. The vast majority of these occur in the descending aorta, and multiple ulcers are not uncommon. Ulcerations with an intimal flap may be distinguished from focal aortic dissections on cross sectional imaging in that the flap extends across the length of the aorta in the latter. Antihypertensive medical therapy and close monitoring is the most common treatment course. For larger and/or deeper ulcers, surgical or endovascular therapy may be pursued.', 'history': 'Patient with a history of hypertension and atherosclerosis.', 'imagePoolId': '947416de-a16e-47b7-8319-3be51e8bc7a2', 'name': 'Abdominal aortic ulceration', 'teachingPoint': None, 'demographics': '75 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*PA chest radiograph in a patient with penetrating aortic ulcer (PAU) of the aortic arch shows a mediastinal mass lateral to the aortic arch from associated hematoma and a left pleural effusion . Note the aortic intimal calcification .*
+
+
+*PA chest radiograph in a patient with penetrating aortic ulcer (PAU) of the aortic arch shows a mediastinal mass lateral to the aortic arch from associated hematoma and a left pleural effusion . Note the aortic intimal calcification .*
+
+
+*Sagittal oblique CTA in the same patient shows extensive atherosclerosis of the aorta with a small, penetrating ulcer and a large focal mural hematoma . PAUs are associated with a variable degree of intramural hematoma (IMH), either focal (as in this case) or diffuse.*
+
+
+*Axial CECT of the chest in a patient with PAU at the proximal ascending thoracic aorta shows an outpouching extending beyond the aortic wall , adjacent atherosclerosis , and bilateral pleural effusions .*
+
+
+*Coronal oblique CECT in the same patient shows the relationship of PAU with the ascending aorta and left coronary artery . Location in the ascending aorta and acute chest pain are the most important factors that determine emergent treatment due to high risk of rupture.*
+
+
+*Axial NECT of the chest in a patient with PAU at the aortic arch with aortic rupture shows an anterior mediastinal mass due to mediastinal hematoma , indicating rupture. Note that PAU can simulate the presence of an anterior mediastinal mass, and contrast remains critical for appropriate differentiation.*
+
+
+*Axial CTA of the chest in the same patient shows a large ulceration along the anterior proximal aortic arch with surrounding mediastinal hemorrhage and extensive atherosclerosis .*
+
+
+*Sagittal oblique reformation in the same patient depicts the ulceration and large pseudoaneurysm along the proximal arch. Note extensive calcific atherosclerosis .*
+
+
+*Volume-rendered CTA in the same patient shows the large pseudoaneurysm , which is compressing the proximal left carotid artery . Volume renderings may be helpful to thoracic surgeons for surgical planning.*
+
+
+*SSFP (left) and T1W (middle) MR images in a 67-year-old woman with chest pain and history of anaphylaxis with iodinated contrast shows a small PAU in the descending thoracic aorta with surrounding IMH . Angiogram prior to stent placement (right) shows the PAU . (Courtesy S. Kligerman, MD.)*
+
+
+*CTA in a man with chest pain shows extensive atherosclerotic disease and a small PAU with surrounding IMH . (Courtesy S. Kligerman, MD.)*
+
+
+*Axial CECT of the chest in a patient with PAU at the descending thoracic aorta shows lobulation of the right lateral wall of the aorta with a variable amount of adjacent IMH . The most common location of PAU is the descending aorta, followed by the abdominal aorta, and, rarely, the ascending aorta.*
+
+
+*Coronal oblique CECT in the same patient shows focal outpouching from PAU with a small amount of adjacent IMH . Also note scattered atherosclerosis .*
+
+
+*Axial NECT of the chest in a patient with IMH secondary to PAU shows crescentic hyperdensity along the descending aorta, consistent with IMH. NECT is helpful in differentiating PAU with IMH from arteritis, which is not hyperdense.*
+
+
+*Sagittal CTA of the chest in a patient with IMH secondary to PAU shows a large, relatively shallow ulceration extending beyond the expected aortic margin. Note also the aortic wall thickening from IMH.*
+
+
+*Axial NECT (left) and CTA (right) in a patient with contained rupture of a descending aortic PAU show retrocrural hemorrhage and atherosclerosis with contrast extending beyond the expected aortic margin.*
+
+
+*Axial CTA of the chest before (left) and after (right) treatment shows a PAU along the descending thoracic aorta , which is excluded after placement of an endovascular stent . Endovascular therapy has become the treatment of choice whenever feasible.*
+
+
+### Additional Images
+
+
+*Coronal CECT shows atherosclerosis of the aorta with contrast projecting beyond the expected margin of the aortic wall and adjacent thickening of the aortic wall . The descending aorta is typically involved.*
+
+
+*Oblique catheter angiography shows a small contrast outpouching on the wall of the descending thoracic aorta, representing an aortic ulceration. The aorta seems only mildly diseased on the aortogram.*
+
+
+*DSA in the same patient shows the outpouchings in the abdominal aorta . There is mild common iliac artery atherosclerotic disease and more extensive disease involving the infrarenal aorta.*
+
+
+*Oblique CECT shows a large ulceration in the transverse aortic arch. Additionally, the aorta has mild diffuse disease with scattered atherosclerotic calcifications .*
+
+
+*Axial CECT shows contrast extending through a defect in the mural thrombus that lines the aortic lumen. There is calcification in the aortic wall and an area of soft tissue density outside the aorta , which could represent bleeding.*
+
+
+*DSA shows a focal outpouching of contrast in the descending thoracic aorta. This contrast collection persists after it has almost completely cleared from the remainder of the aorta. This abnormality could be treated with endovascular stent grafting.*
+
+
+*Coronal MR angiogram shows 2 areas of focal outpouching in the distal abdominal aorta. Although penetrating ulcers are uncommon in the abdominal aorta, these areas are suspicious. There is also common iliac artery atherosclerotic disease .*
+
+
+*Axial CECT shows a diseased and ectatic aortic arch with moderate atherosclerotic calcification . There is a focal outpouching of contrast projecting from the lateral aortic wall, which represents a PAU.*
+
+
+*Axial chest CTA in a patient with PAU along the descending thoracic aorta shows a contrast collection extending beyond the expected aortic margin. Note surrounding soft tissue , consistent with focal IMH.*
+
+
+*Sagittal CTA in the same patient shows a well-defined PAU . Note adjacent atherosclerotic plaques . In contrast to mycotic pseudoaneurysm or traumatic aortic injury, PAU is invariably associated with atherosclerosis.*
+
+
+*Axial CTA in a patient with PAU and aneurysm of the descending thoracic aorta with aortic dissection shows an ulceration with conspicuous, thick, intimomedial, flap-like margins in a location not classic for aortic dissection. These are helpful features to differentiate aortic dissection arising from PAU from classic aortic dissection.*
+
+
+*Axial chest CTA in a patient with ulcerated atherosclerotic plaque shows that, as opposed to PAU, the ulcerated plaque does not extend beyond the expected aortic wall margin.*
+
diff --git a/docs_md/articles/pericardial-calcification_102d1a2a-5968-4ccc-bb39-13ea2e395a88.md b/docs_md/articles/pericardial-calcification_102d1a2a-5968-4ccc-bb39-13ea2e395a88.md
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+++ b/docs_md/articles/pericardial-calcification_102d1a2a-5968-4ccc-bb39-13ea2e395a88.md
@@ -0,0 +1,121 @@
+---
+title: "Pericardial Calcification"
+docid: "102d1a2a-5968-4ccc-bb39-13ea2e395a88"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Pericardial Calcification"
+ slug: "pericardial-calcification"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "93cf53a5-85ce-4d16-b488-1398159edb6e"
+imageCount: 9
+lastUpdated: "03/17/22"
+pageDescription: "Pericardial Calcification"
+pageKeywords: "Cardiac, Differential Diagnosis, Pericardial Calcification"
+pageTitle: "Pericardial Calcification | STATdx"
+enhancedTitle: "Pericardial Calcification"
+type: "DDX"
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Pericardial Calcification"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Any cause of chronic or prior pericarditis may cause pericardial calcification
+ - Calcification features or distribution limited in identifying etiology; history most helpful
+ - Calcifications adjacent to pericardium are often mistaken for pericardial calcifications, particularly when overlying left ventricle
+ - Constrictive pericarditis: Pericardial calcification with dilated IVC, SVC, atria, tubular ventricles, and hepatic vein contrast reflux
+ - Reporting spatial location of calcifications assist in planning surgical therapy
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Prior Pericarditis**
+ - Although TB is uncommon in developed world, it is common cause of calcified pericardium in developing world
+ - Characteristically thick, irregular, amorphous calcifications predominantly over anterior and inferior RV: Panzer heart appearance on radiography
+ - Uremic pericarditis: Eggshell calcification pattern
+ - Idiopathic: Diagnosis of exclusion; often result of undiagnosed viral infection
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Prior Hemopericardium**
+ - Most commonly from trauma, malignancy, or surgery
+ - Metastasis
+ - Far more common than primary tumors
+ - Nodular pericardium with coexistent hemopericardium
+ - Lung cancer, breast cancer, and lymphoma account for 75% of cases
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Prior Radiation Therapy**
+ - Acute and chronic forms of radiation pericarditis can lead to calcification
+ - Radiotherapy exceeding 40 Gy, dose commonly delivered for Hodgkin disease or lung cancer
+ - Acute pericarditis can occur weeks to months after radiation
+ - Acute pericarditis is generally symptomatic
+ - Chronic pericarditis does not occur before 6 months
+ - More often leads to constrictive physiology but can be asymptomatic
+ - Fibrosis of adjacent mediastinal adipose tissue
+
+
+## Images
+
+
+### Selected Images
+
+
+**Prior Pericarditis**
+*Axial CECT shows dense pericardial calcifications of the pericardium . Cardiac chambers appear normal in size. Compare with postsurgical appearance in next image.*
+
+
+**Prior Pericarditis**
+*Axial CECT shows dense pericardial calcifications of the pericardium . Cardiac chambers appear normal in size. Compare with postsurgical appearance in next image.*
+
+
+**Prior Pericarditis**
+*Axial CECT in the same patient after pericardial stripping is shown. Cardiac enlargement illustrates the degree of anatomic distortion caused by pericardial constriction.*
+
+
+**Prior Pericarditis**
+*Axial NECT shows pericardial thickening in a patient with chronic pericarditis due to rheumatoid arthritis. Chronic pericarditis can lead to calcifications.*
+
+
+**Prior Pericarditis**
+*Axial CECT in the same patient 10 years later shows development of pericardial calcifications in region of longstanding pericardial thickening. Note focal constriction on right ventricle .*
+
+
+**Prior Pericarditis**
+*Axial CECT shows apical predominant pericardial calcifications in a 45-year-old patient with history of tuberculosis. Note tubular ventricles and dilated atria .*
+
+
+**Prior Pericarditis**
+*Lateral radiograph shows pericardial calcifications of the anterior and inferior wall in a patient with suspected remote pericarditis.*
+
+
+**Prior Hemopericardium**
+*Axial NECT shows high-density pericardial fluid in a patient with hemopericardium. Although this represents the acute presentation, these patients may develop pericardial calcifications.*
+
+
+**Prior Radiation Therapy**
+*Short-axis black-blood MR shows signal loss at the anterior pericardium in a patient with focal pericardial calcification from prior mediastinal radiation. Signal loss is greater with gradient echo sequences as opposed to the spin echo sequence shown here.*
+
+
+### Additional Images
+
+
+**Prior Radiation Therapy**
+*Axial CT shows pericardial fluid and thickening in a patient who recently received radiation. Note inflammatory stranding of the chest wall adjacent to the pericardium . These patients may progress to develop pericardial calcifications.*
+
diff --git a/docs_md/articles/pericardial-mass_39979947-4afb-4370-86ed-6b6d196bca78.md b/docs_md/articles/pericardial-mass_39979947-4afb-4370-86ed-6b6d196bca78.md
new file mode 100644
index 0000000..d5b0c8a
--- /dev/null
+++ b/docs_md/articles/pericardial-mass_39979947-4afb-4370-86ed-6b6d196bca78.md
@@ -0,0 +1,123 @@
+---
+title: "Pericardial Mass"
+docid: "39979947-4afb-4370-86ed-6b6d196bca78"
+authors:
+ - key: "df804626-c042-4296-96e3-836a6da50fd6"
+ value: "Gregory Kicska, MD, PhD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "952326a0-b3ea-4a21-aa7a-d796cc9325ed"
+ -
+ name: "Pericardial Mass"
+ slug: "pericardial-mass"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "bd8780ab-7d7b-4c9d-b43e-674a7871c2c3"
+imageCount: 9
+lastUpdated: "03/17/22"
+pageDescription: "Pericardial Mass"
+pageKeywords: "Cardiac, Differential Diagnosis, Pericardial Mass"
+pageTitle: "Pericardial Mass | STATdx"
+enhancedTitle: "Pericardial Mass"
+type: "DDX"
+breadcrumbs:
+ - "Cardiac"
+ - "Differential Diagnosis"
+ - "Pericardial Mass"
+---
+# ESSENTIAL INFORMATION
+
+- ## Key Differential Diagnosis Issues
+
+
+ - Diagnostic evaluation should focus on distinction between neoplastic and nonneoplastic etiology
+ - Loculated fluid can mimic neoplastic pericardial mass
+ - Focal thickening is equally likely to be metastatic tumor vs. other cause if patient has history of breast cancer, lung cancer, or lymphoma
+ - Without history of cancer, undiagnosed malignancy less likely
+ - Absence of enhancement and low-density fluid suggest nonneoplastic etiology
+- ## Helpful Clues for Common Diagnoses
+
+
+ - **Metastatic Disease**
+ - Far more common than primary tumors
+ - Nodular, enhancing pericardium; mediastinal adenopathy
+ - Lung, breast, and lymphoma account for 75% of cases
+ - May present with hemopericardium
+ - **Loculated Fluid or Focal Thickening**
+ - Low-density, well-circumscribed fluid suggests pericardial cyst
+ - Thick, enhancing wall surrounding fluid is seen with abscess
+- ## Helpful Clues for Less Common Diagnoses
+
+
+ - **Benign Primary Pericardial Tumors**
+ - **Teratoma**: Most common benign tumor, heterogeneous CT attenuation
+ - Most often in children
+ - **Lipoma**: Encapsulated fat, high T1 signal decreased with fat suppression
+ - **Hemangioma**: Strong contrast enhancement
+ - **Fibroma**: Low T1 and T2 signal; no contrast enhancement
+- ## Helpful Clues for Rare Diagnoses
+
+
+ - **Primary Pericardial Mesothelioma**
+ - Most common primary neoplasm of pericardium, but rare
+ - Represents 50% of all primary pericardial tumors, but only 1% of all malignant mesothelioma
+ - Diffuse circumferential nodular pericardial thickening with calcification and associated effusion; FDG avid
+ - **Other Malignant****Primary Pericardial Tumors**
+ - Lymphoma, sarcoma, and liposarcoma most common histologies
+ - Large, enhancing mass associated with hemopericardium
+
+
+## Images
+
+
+### Selected Images
+
+
+**Metastatic Disease**
+*Axial CECT shows nodular, enhancing pericardial masses in a patient with known metastatic lung cancer. These can present with hemopericardium.*
+
+
+**Metastatic Disease**
+*Axial CECT shows nodular, enhancing pericardial masses in a patient with known metastatic lung cancer. These can present with hemopericardium.*
+
+
+**Loculated Fluid or Focal Thickening**
+*Frontal radiograph shows a rounded opacity superimposed upon the right heart border . On CT, this was found to represent a pericardial cyst.*
+
+
+**Loculated Fluid or Focal Thickening**
+*Axial CECT shows a pericardial cyst as a well-circumscribed, thin-walled fluid collection adjacent to the right atrium without adjacent inflammatory stranding.*
+
+
+**Loculated Fluid or Focal Thickening**
+*Axial CECT in a patient with a pericardial abscess shows a fluid collection adjacent to the right atrial appendage . The collection has thick, enhancing walls, and there is associated pericardial thickening and inflammatory stranding.*
+
+
+**Hemangioma**
+*Coronal CECT in a patient with pericardial hemangioma shows heterogeneous contrast enhancement of a pericardial mass adjacent to the left atrial appendage . Fat planes adjacent to the pericardium are preserved, a feature more commonly associated with benign etiology.*
+
+
+**Metastatic Disease**
+*Axial T2 FS MR shows high signal adjacent to the left atrial appendage in a patient with a hemangioma. Note the aorta as an anatomic landmark .*
+
+
+**Other Malignant Primary Pericardial Tumors**
+*Axial CECT shows extension of a pleural mesothelioma along the anterior pericardium . Pericardial extension of a primary pleural mesothelioma is far more common than a primary pericardial tumor.*
+
+
+**Other Malignant Primary Pericardial Tumors**
+*Axial CECT in a patient with lymphoma shows a right-sided heart mass that extends from the pericardium to the right ventricle lumen . Note the hemopericardium .*
+
+
+### Additional Images
+
+
+**Benign Primary Pericardial Tumors**
+*Axial NECT shows well-circumscribed mixed fat and soft tissue adjacent to the pericardium . This was thought to be a teratoma.*
+
diff --git a/docs_md/articles/persistent-fifth-arch_931c64bc-e8a0-4a99-848b-1429f3c1d500.md b/docs_md/articles/persistent-fifth-arch_931c64bc-e8a0-4a99-848b-1429f3c1d500.md
new file mode 100644
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--- /dev/null
+++ b/docs_md/articles/persistent-fifth-arch_931c64bc-e8a0-4a99-848b-1429f3c1d500.md
@@ -0,0 +1,253 @@
+---
+title: "Persistent Fifth Arch"
+docid: "931c64bc-e8a0-4a99-848b-1429f3c1d500"
+authors:
+ - key: "9fea2857-d729-4fe4-b4fd-3b7bf1db23cf"
+ value: "Mortadha Al-Kinani, MD, MBChB"
+ - key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
+ value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Persistent Fifth Arch"
+ slug: "persistent-fifth-arch"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "2c81f654-df79-4360-b8b4-b5042f45e8e5"
+imageCount: 4
+lastUpdated: "01/24/25"
+pageDescription: "Persistent Fifth Arch"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Persistent Fifth Arch"
+pageTitle: "Persistent Fifth Arch | STATdx"
+enhancedTitle: "Persistent Fifth Arch"
+type: "DX"
+references: true
+cases: 1
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Persistent Fifth Arch"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Rare congenital vascular anomaly
+ - May be isolated or associated with other abnormalities
+ - Complex congenital cardiac heart disease
+ - Vascular anomalies
+ - Skeletal anomalies
+ - 2 distinct forms
+ - **Systemic-to-systemic connection**: 5th arch arises at brachiocephalic trunk and reconnects at descending aorta
+ - **Systemic-to-pulmonary connection**: 5th arch connects with embryologic remnant of 6th aortic arch, which is usually left pulmonary artery
+- ## Imaging
+
+
+ - Contrast-enhanced MRA most appropriate in children with suspected persistent 5th arch
+ - Short segment of duplication aortic arch with 2 parallel distinct lumina in systemic-to-systemic connection
+ - Abnormal vessel connecting aorta with isolated pulmonary artery in systemic-to-pulmonary connection
+- ## Clinical Issues
+
+
+ - Presentation depends on which type of connections exists and on associated cardiac and vascular anomalies
+ - Associated cardiovascular anomalies
+ - Ventricular septal defect (most common)
+ - Pulmonic valve or artery stenosis/atresia
+ - Interruption of aortic arch
+ - Coarctation of aorta
+ - Transposition of great arteries
+ - Pentalogy of Fallot
+ - Patent ductus arteriosus
+ - Tricuspid atresia
+ - In case of coarctation/obstruction, surgical patching or conduit interposition may be indicated
+
+# TERMINOLOGY
+
+- ## Synonyms
+
+
+ - Ipsilateral double aortic arch
+ - Double lumen aortic arch
+- ## Definitions
+
+
+ - Rare congenital vascular anomaly of aortic arch
+ - May be isolated
+ - Often associated with other congenital cardiac, vascular, or skeletal anomalies
+ - 2 distinct forms
+ - Systemic-to-systemic connection
+ - 5th arch arises at brachiocephalic trunk and reconnects at descending aorta
+ - Systemic-to-pulmonary connection
+ - 5th arch connects with embryologic remnant of 6th aortic arch, which is usually left pulmonary artery
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Short segment of duplication of aortic arch with 2 parallel distinct lumina
+ - May have interrupted arch
+ - Abnormal vessel connecting aorta with pulmonary artery
+ - ### Location
+
+
+ - Aortic arch
+ - Cephalad of arches is 4th arch, which gives rise to arch vessels
+ - Lower arch is persistent 5th arch
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - May demonstrate associated findings, such as vertebral anomalies
+- ## CT Findings
+
+
+ - ### CTA
+
+
+ - 2 distinct left aortic arches with what may appear as septation separating them
+ - Double-barrel appearance on coronal oblique short-axis views
+ - May have interrupted arch
+ - May show anomalous connection between aorta and isolated left pulmonary artery
+ - May demonstrate associated cardiovascular and skeletal abnormalities
+- ## MR Findings
+
+
+ - Same as CTA findings
+ - Superior to echocardiography due to acoustic window restrictions near aortic arch
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - CTA or MRA
+ - ### Protocol advice
+
+
+ - Contrast-enhanced MRA most appropriate in children with suspected persistent 5th arch
+
+# DIFFERENTIAL DIAGNOSIS
+
+- [Aortic Dissection](/document/aortic-dissection/57e3428e-1f18-4f38-95c6-f7fe2d93c00a)
+ - Easily differentiated by double-barrel appearance of persistent 5th arch: 2 round lumina form "figure of 8" on arch short-axis views
+- [Patent Ductus Arteriosus](/document/patent-ductus-arteriosus/5ba3261d-bd13-4542-92ec-5db5274e2050)
+ - Systemic-to-pulmonary connection may mimic large PDA
+ - PDA would communicate distally to arch arteries
+- ## Aortopulmonary Window
+
+
+ - Abnormal connection between proximal aorta and pulmonary trunk
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Often presents soon after birth due to associated cardiac or vascular defects
+ - Ventricular septal defect
+ - Pulmonic valve or artery stenosis/atresia
+ - Interruption of aortic arch
+ - Complex congenital heart disease
+ - Often present after birth due to associated cardiac or vascular defects
+ - Ventricular septal defect (most common), pulmonic valve or artery stenosis/atresia, tricuspid atresia
+ - Interruption of aortic arch, coarctation of aorta, transposition of great arteries, persistent truncus arteriosus, PDA
+ - Could be hemodynamically beneficial
+ - Systemic-to-systemic connection; if associated with coarctation of aorta or interrupted aortic arch
+ - Systemic-to-pulmonic connection; when associated with pulmonary or tricuspid atresia
+ - ### Clinical profile
+
+
+ - Association with intrauterine thalidomide exposure and chromosomal disorders
+ - Weinberg classification defines 3 types (A,B,C)
+ - Type A: Double-lumen aortic arch (arch vessels arise from upper 4th; lower is 5th arch)
+ - Type B: Single-lumen arch; 4th arch is interrupted; 5th arch originates from ascending and connects to descending aorta
+ - Type C: 5th originates from proximal brachiocephalic artery off of ascending aorta and connecting to pulmonary artery via 6th arch
+ - Associated cardiovascular anomalies include
+ - Coarctation
+ - Pulmonary atresia or stenosis
+ - Transposition of great arteries
+ - Truncus arteriosus
+ - Pentalogy of Fallot
+ - PDA, ventricular septal defect
+ - Tricuspid atresia
+- ## Demographics
+
+
+ - ### Epidemiology
+
+
+ - Extremely rare congenital malformation
+- ## Treatment
+
+
+ - In case of coarctation/obstruction, surgical patching or conduit interposition may be indicated
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - May have obstruction due to associated coarctation
+ - Check BP difference between upper and lower extremities (BP in both arms)
+
+ 597b11ca-b733-4d2c-8265-416a2b493b11
+
+## References
+
+# Selected References
+
+1. [Shan H et al: Persistent fifth aortic arch: a comprehensive literature review. Front Pediatr. 11:1183345, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37435167%5Bpmid%5D)
+1. [Liu Y et al: Persistent fifth aortic arch: a single-center experience, case series. Transl Pediatr. 10(6):1566-72, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34295771%5Bpmid%5D)
+1. [Kligerman S et al: Persistent fifth aortic arch in a patient with a history of intrauterine thalidomide exposure. J Cardiovasc Comput Tomogr. 3(6):412-4, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19709946%5Bpmid%5D)
+1. [Kirsch J et al: Magnetic resonance angiography of an ipsilateral double aortic arch due to persistent left fourth and fifth aortic arches. Pediatr Radiol. 37(5):501-2, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17415604%5Bpmid%5D)
+1. [Zhao YH et al: Surgical treatment of persistent fifth aortic arch associated with interrupted aortic arch. Ann Thorac Surg. 84(3):1016-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17720425%5Bpmid%5D)
+1. [Zhong Y et al: Contrast-enhanced magnetic resonance angiography of persistent fifth aortic arch in children. Pediatr Radiol. 37(3):256-63, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17200843%5Bpmid%5D)
+1. [Hwang MS et al: Isolated persistent fifth aortic arch with systemic-to-pulmonary arterial connection. J Thorac Cardiovasc Surg. 126(5):1643-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14666049%5Bpmid%5D)
+
+## Cases
+
+- {'cases': [{'authors': [{'key': 'b00d2bdb-66e1-41ed-90b4-c52904f4d598', 'value': 'Seth Kligerman, MD, MS'}], 'caseVersionId': '33e370c1-f5d0-4136-8a26-f4f065378eab', 'description': "Coronal CT angiograms through the chest (Figs. 1-3) and sagittal oblique volume-rendered image (Fig. 4) with a posterior view of the aorta show the transverse aorta bifurcating into the superior and inferior channels just proximal to the level of the left brachiocephalic artery (, Fig. 4) and again merging just distal to the left subclavian artery (, Figs. 3-4). The more inferior arch is the persistent 5th arch. All of the arch vessels, including the left common carotid artery (, Figs. 1, 4,), arise from the true arch. Volume-rendered 3-D cine clip (Vid. 1) again shows the bifurcation.", 'history': 'Patient with multiple congenital bony anomalies and a history of intrauterine thalidomide exposure undergoes preoperative CT.', 'imagePoolId': '8123c1a9-ea90-4684-985e-d9c873cc0c2d', 'name': 'Persistent Fifth Arch', 'teachingPoint': 'Persistence of the 5th aortic arch is an exceedingly rare anomaly. There are 2 mains forms. In the systemic to systemic form, the arch connects the ascending and descending aorta. In the systemic to pulmonary form, the 5th arch connects to a pulmonary artery, usually the left.', 'demographics': '45 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Aortic arch "candy cane" view of CTA shows separation of the aortic arch into 2 distinct vessels (*). The superior is the normal 4th aortic arch giving rise to the arch vessels. The inferior is the persistent 5th aortic arch.*
+
+
+*Aortic arch "candy cane" view of CTA shows separation of the aortic arch into 2 distinct vessels (*). The superior is the normal 4th aortic arch giving rise to the arch vessels. The inferior is the persistent 5th aortic arch.*
+
+
+*Coronal CTA in the same patient shows the short axis of aortic arches with a double-barrel appearance (*). The 4th and persistent 5th arch have a figure of 8 configuration in the short axis, which allows differentiation from dissection.*
+
+
+*Oblique 3D volume-rendered reconstruction shows the relationship of the 4th arch with the arch vessels. Note the abnormal persistent 5th arch arising from the aorta at the level of the brachiocephalic trunk and reentering into the descending thoracic aorta at its isthmus.*
+
+
+*Oblique 3D reconstruction of the skull in the same patient shows a cleft palate . Other skeletal anomalies in this patient include fused ribs and hemi vertebra (not shown).*
+
diff --git a/docs_md/articles/pseudocoarctation_a5a7c623-d5cb-4bb1-b628-986d9ca1f94a.md b/docs_md/articles/pseudocoarctation_a5a7c623-d5cb-4bb1-b628-986d9ca1f94a.md
new file mode 100644
index 0000000..615ae07
--- /dev/null
+++ b/docs_md/articles/pseudocoarctation_a5a7c623-d5cb-4bb1-b628-986d9ca1f94a.md
@@ -0,0 +1,428 @@
+---
+title: "Pseudocoarctation"
+docid: "a5a7c623-d5cb-4bb1-b628-986d9ca1f94a"
+authors:
+ - key: "a354e6da-2757-40e8-b7ff-5e6fb6413ff6"
+ value: "Sachin S. Saboo, MD, FRCR, FSCMR"
+ - key: "b00d2bdb-66e1-41ed-90b4-c52904f4d598"
+ value: "Seth Kligerman, MD, MS"
+ - key: "0e97d53b-518f-493d-bcf9-236f6494f4c2"
+ value: "Carlos A. Rojas, MD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Pseudocoarctation"
+ slug: "pseudocoarctation"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "bfaa0bcc-9296-4ce2-8fd6-7aefa8d90588"
+imageCount: 23
+lastUpdated: "01/24/25"
+pageDescription: "Pseudocoarctation"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Pseudocoarctation"
+pageTitle: "Pseudocoarctation | STATdx"
+enhancedTitle: "Pseudocoarctation"
+type: "DX"
+references: true
+ddx: true
+cases: 1
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Pseudocoarctation"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Aortic arch elongation with kinking of thoracic aorta distal to origin of left subclavian artery at level of ductus arteriosus
+- ## Imaging
+
+
+ - Frontal chest radiograph
+ - Mass-like opacity in left superior mediastinum; may mimic mediastinal mass
+ - Double left aortic arch and reverse 3 sign
+ - No rib notching
+ - Lateral chest radiograph
+ - Redundant aortic arch buckled forward at isthmus
+ - Best imaging tool: Contrast-enhanced 3D CT and MR angiography, phase-contrast CMR
+ - Elongated distal aortic arch and proximal descending thoracic aorta with kinking and buckling
+ - Evaluate for complications: Aneurysm formation, subclavian steal syndrome
+ - Pseudocoarctation of aorta (PCOA) distinguished from coarctation of aorta (COA) by
+ - No hemodynamically significant aortic narrowing by MR or catheter angiography
+ - No collateral arteries or significant poststenotic dilatation
+ - No left ventricular hypertrophy
+- ## Top Differential Diagnoses
+
+
+ - Coarctation of aorta
+ - Aortic aneurysm
+ - Mediastinal mass
+- ## Pathology
+
+
+ - Elongation of distal aortic arch due to abnormal growth of preductal aorta
+- ## Diagnostic Checklist
+
+
+ - Sagittal views for CTA and MRA are most useful for demonstrating PCOA
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Pseudocoarctation of aorta (PCOA)
+- ## Synonyms
+
+
+ - Aortic buckling
+ - Aortic kinking
+ - Atypical coarctation
+ - Nonobstructive coarctation
+ - Redundant aortic arch
+- ## Definitions
+
+
+ - Elongation and kinking of aortic arch and proximal thoracic aorta distal to origin of left subclavian artery (SCA) at level of ductus arteriosus
+ - Distinguished from coarctation of aorta (COA) by lack of hemodynamically significant stenosis
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Kinking and buckling of aorta at level of ductus arteriosus with pressure gradient < 25 mmHg
+ - ### Location
+
+
+ - Aortic isthmus at site of attachment of ligamentum arteriosum distal to origin of left SCA
+ - ### Size
+
+
+ - Normal caliber or dilatation > 4 cm, or, occasionally, stenotic at site of aortic buckling
+ - ### Morphology
+
+
+ - Elongation of distal aortic arch (AA) and proximal descending thoracic aorta (DTA); acute anterior angulation of AA at level of ligamentum arteriosum without significant obstruction
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - CTA or MRA
+ - ### Protocol advice
+
+
+ - 3D CTA in sagittal orientation with MIP and MPR reconstructions
+ - 3D high-resolution MRA in sagittal view with MIP and volume rendering
+ - Time-resolved MRA for evaluation of flow patterns and collateral pathways
+ - Phase-contrast velocity-encoded sequence for measurement of peak velocities and pressure gradients
+ - Axial CT/MR useful for confident evaluation of coexisting venous anomaly
+ - Short-axis images useful for evaluation of coexisting bicuspid valve
+ - 4D flow MR for flow patterns and quantification
+- ## Radiographic Findings
+
+
+ - Frontal chest radiograph
+ - Mediastinal widening, mass-like opacity in left superior mediastinum mimicking mediastinal mass or aneurysm
+ - Double left AA sign
+ - Aorta proximal to kinking appears higher than normal AA
+ - Aorta distal to kinking appears lower than normal with tortuous AA course, producing S-shaped figure
+ - Reverse 3 sign: Outlines medial side of aortic indentation in DTA and E sign in esophagogram
+ - No rib notching
+ - Lateral chest radiograph
+ - AA is buckled forward at isthmus
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - Elongated and tortuous distal AA and proximal DTA
+ - Anterior and medial displacement of distal AA
+ - Kink in posterior and lateral margins of aorta at isthmus
+ - ### CTA
+
+
+ - Elongated redundant distal AA and proximal DTA with kinking and buckling
+ - Minimal or no luminal narrowing of distal transverse arch at attachment of ligamentum arteriosum
+ - High AA extending into left supraclavicular region (children)
+ - Bicuspid aortic valve may be present
+ - Aortic aneurysm may be present
+ - Poststenotic dilatation ± depending on hemodynamic significance
+ - Abnormal origins of arch arteries; dilatation of brachiocephalic arteries
+ - General
+ - Distinguished from COA based on absent hemodynamically significant aortic narrowing, poststenotic dilatation, collateral arteries, and left ventricular hypertrophy
+- ## MR Findings
+
+
+ - ### MRA
+
+
+ - Contrast-enhanced 3D MRA
+ - Kinking and buckling of elongated and tortuous distal AA and proximal DTA
+ - May be associated with aortic aneurysms due to altered hemodynamics
+ - Time-resolved MRA
+ - May show steal phenomenon in presence of SCA stenosis as reversal of flow in vertebral artery
+ - Phase-contrast flow MR
+ - No elevation of velocity; normal/minimal increased pressure gradient (peak pressure gradient < 25 mmHg) across kink
+ - Flow reversal in steal phenomenon
+ - 4D flow CMR/4D phase-contrast CMR
+ - Single 3D acquisition volume with accurate retrospective flow calculation through any plane
+ - Quantification of flow volume, retrograde flow/fraction in aorta, peak velocity and gradient at site of maximum kinking
+ - 3D flow visualization of highly disrupted flow patterns in tortuous aorta
+ - Superior spatial coverage; better at capturing peak velocity of stenotic jet, which is absent in PCOA
+ - Elevated wall shear stress (WSS) in PCOA may contribute to aorta dilatation or pseudoaneurysm due to its correlation with blood flow velocity
+ - Elevated flow velocity and elevated peak WSS seen in kinked aorta/ PCOA
+ - Aneurysm sacs associated with PCOA shows vortex flow during systole with lower peak WSS
+ - General: Distinguished from COA based on absence of hemodynamically significant aortic narrowing, significant poststenotic dilatation, left ventricular hypertrophy, and collateral arteries
+- ## Angiographic Findings
+
+
+ - High position of AA
+ - Reverse 3 sign: Notch in descending aorta at attachment of short ligamentum arteriosum
+ - Gold standard for accurate pressure gradient measurement before intervention planning or if diagnostic uncertainty
+
+# DIFFERENTIAL DIAGNOSIS
+
+- [Coarctation of Aorta](/document/coarctation-of-aorta/c0b23d8c-05e3-4373-b5d9-2de1590414a7)
+ - Congenital narrowing of aorta at isthmus distal to left SCA origin
+ - Diffuse hypoplasia of AA distal to origin of innominate artery may be associated
+ - Chest radiograph with rib notching or reverse E or 3 sign from pre- and poststenotic dilatation
+ - Hemodynamically significant stenosis
+ - Elevated peak pressure gradient > 20 mmHg
+ - Poststenotic aortic dilation
+ - Collateral vessels: Internal mammary, intercostal, parascapular, epigastric arteries
+ - Rib notching on chest XR
+ - Left ventricular hypertrophy
+ - Both COA and PCOA associated with bicuspid aortic valve
+- ## Hypoplastic Aortic Arch
+
+
+ - Mostly in children; commonly seen in patients with COA
+ - If external diameter of distal arch segment is < 50% of ascending aorta; z-score of 2 or lower, no pressure gradient across narrowed portion
+- ## Aortic Aneurysm
+
+
+ - Usually seen in atherosclerotic aorta with calcified intimal plaque
+ - Saccular or fusiform dilatation with mural thrombus often present within periphery of aneurysm
+ - Commonly seen in older adult patients; may rupture or result in aortic dissection
+- ## Mediastinal Mass
+
+
+ - Mass-like opacity on chest radiograph
+ - CT and MR angiography can differentiate soft tissue mass from PCOA
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Elongated distal AA and proximal DTA due to failed compression of 3rd-7th dorsal aortic segments causing longer AA that kinks at ductus arteriosum level
+ - Short taut ligamentum arteriosum or patent ductus arteriosus
+ - ### Associated abnormalities
+
+
+ - Aberrant SCA; cervical AA; left superior vena cava, left vertebral artery origin from AA, aneurysmal dilatation of SCA
+ - Aortic stenosis; sinus of Valsalva aneurysm; coarctation of distal descending aorta
+ - Bicuspid aortic valve; aortic valve incompetence; mitral valve prolapse
+ - Left-to-right shunts; atrial septal defect; ventricular septal defect; patent ductus arteriosus
+ - Aortic aneurysm leading to sudden aortic rupture or aortic dissection
+- ## Microscopic Features
+
+
+ - Aneurysms associated with PCOA result from cystic medial necrosis rather than atherosclerosis
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - Usually asymptomatic or resistant or difficult to treat hypertension
+ - ### Other signs/symptoms
+
+
+ - Symptoms related to complications
+ - Aneurysm; may be asymptomatic or shortness of breath, hoarseness, and dysphagia due to compression
+ - Aneurysm rupture or dissection with chest and back pain, shortness of breath, hypotension and hemothorax
+ - Subclavian steal syndrome due to SCA stenosis
+ - Blood pressure discrepancy between upper extremities; dizziness, vertigo, and syncope
+ - Symptoms related to associated abnormalities; dysphagia from compression of esophagus
+ - Extreme caution needed while navigating PCOA for endovascular procedure, such as transcatheter aortic valve implantation (TAVI), to prevent aorta perforation
+- ## Demographics
+
+
+ - ### Epidemiology
+
+
+ - Very uncommon congenital anomaly occurring in isolation or with other congenital heart diseases
+- ## Natural History & Prognosis
+
+
+ - Typically asymptomatic
+ - Aneurysmal dilatation may develop; may result in rupture or dissection
+ - Necessitates annual surveillance of thoracic aorta for early diagnosis and intervention of aortic aneurysm
+- ## Treatment
+
+
+ - Conservative management in asymptomatic and mildly symptomatic patients
+ - Surgical treatment for complications
+ - Aneurysm formation
+ - Open repair: Artificial or biologic grafts
+ - Closed repair: Endovascular stent graft
+ - Aortic dissection
+ - Stanford type A: Surgery due to involvement of ascending aorta
+ - Stanford type B: Medical control of hypertension is standard; surgery or endovascular stenting in complicated cases
+ - Subclavian steal syndrome
+ - Angioplasty/stenting of SCA
+ - Common carotid artery-to-SCA bypass, innominate artery-to-SCA bypass, or axillary artery-to-axillary artery bypass
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Image Interpretation Pearls
+
+
+ - Sagittal views for CTA and MRA are most useful for demonstrating PCOA
+ - 3D CTA with MIP and MPR reconstructions
+ - 3D MRA with MIP and volume rendering
+ - No hemodynamically significant aortic narrowing, poststenotic dilatation, left ventricular hypertrophy, or collateral arteries
+ - Allows differentiation from COA
+
+ f099114d-91fa-442b-983b-31cd9d03f71b
+
+## References
+
+# Selected References
+
+1. [Mahadevappa M et al: Pseudocoarctation of the arch and the abdominal aorta: a review. Curr Cardiol Rev. 19(5):73-82, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36999416%5Bpmid%5D)
+1. [Ito H et al: Assessment of pseudocoarctation of the aorta with saccular aneurysms by four-dimensional flow magnetic resonance imaging and histological analysis. Ann Vasc Dis. 15(4):348-51, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36644272%5Bpmid%5D)
+1. [Dyverfeldt P et al: 4D flow cardiovascular magnetic resonance consensus statement. J Cardiovasc Magn Reson. 17:72, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26257141%5Bpmid%5D)
+1. [Singh S et al: Hypoplasia, pseudocoarctation and coarctation of the aorta - a systematic review. Heart Lung Circ. 24(2):110-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25442062%5Bpmid%5D)
+1. [Panoulas VF et al: Unanticipated pseudocoarctation highlights the importance of visualizing aortic arch anatomy before transfemoral transcatheter aortic valve implantation. Circ Cardiovasc Interv. 7(4):631-3, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25139090%5Bpmid%5D)
+1. [Kimura K et al: Pseudocoarctation of the aorta complicated by thoracic aortic aneurysm. Asian Cardiovasc Thorac Ann. 19(3-4):265-7, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21885555%5Bpmid%5D)
+1. [Bolen MA et al: Pseudocoarction of the aorta and crossed fused ectopic kidney assessed by multidetector computed tomography. J Cardiovasc Comput Tomogr. 4(6):405-6, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=21030333%5Bpmid%5D)
+1. [Rao B et al: Pseudocoarctation with saccular aneurysms, left sided SVC and aberrant right subclavian artery - a case report. J Radiol Case Rep. 4(7):29-33, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=22470744%5Bpmid%5D)
+1. [Ohnuki M et al: [Thoracic aortic aneurysm associated with pseudocoarctation; report of a case.] Kyobu Geka. 62(7):583-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19588831%5Bpmid%5D)
+1. [Son JS et al: Pseudocoarctation of the aorta associated with the anomalous origin of the left vertebral artery: a case report. Korean J Radiol. 9(3):283-5, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18525233%5Bpmid%5D)
+1. [Adaletli I et al: Pseudocoarctation. Can J Cardiol. 23(8):675-6, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17593995%5Bpmid%5D)
+1. [Matsui H et al: Anatomy of coarctation, hypoplastic and interrupted aortic arch: relevance to interventional/surgical treatment. Expert Rev Cardiovasc Ther. 5(5):871-80, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17867917%5Bpmid%5D)
+1. [Tanju S et al: Right cervical aortic arch and pseudocoarctation of the aorta associated with aneurysms and steal phenomena: US, CTA, and MRA findings. Cardiovasc Intervent Radiol. 30(1):146-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=16802075%5Bpmid%5D)
+1. [Choi BW et al: Magnetic resonance angiography of pseudocoarctation. Heart. 90(10):1213, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15367527%5Bpmid%5D)
+1. [Sebastià C et al: Aortic stenosis: spectrum of diseases depicted at multisection CT. Radiographics. 23 Spec No:S79-91, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14557504%5Bpmid%5D)
+1. [Taneja K et al: Pseudocoarctation of the aorta: complementary findings on plain film radiography, CT, DSA, and MRA. Cardiovasc Intervent Radiol. 21(5):439-41, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9853156%5Bpmid%5D)
+1. [Lajos TZ et al: Pseudocoarctation of the aorta: a variant or an entity? Chest. 58(6):571-6, 1970](http://www.ncbi.nlm.nih.gov/pubmed/?term=5486551%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Narrowed Aorta
+DDX:763503a4-a7b8-4aff-8846-3dfbe312125c
+
+## Cases
+
+- {'cases': [{'authors': [{'key': None, 'value': None}, {'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}, {'key': '3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1', 'value': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI'}], 'caseVersionId': '46419090-2276-43b0-af99-f8af72b59c4c', 'description': 'Sagittal oblique VR image from contrast enhanced CTA (#1) shows a redundant morphology of the aortic arch with kinking and mild narrowing (arrows) of the proximal descending aorta at the level of the ligamentum arteriosum, highly suggestive of pseudocoarctation. No enlarged collateral bronchial, intercostal, or internal mammary arteries are present, which suggests that the aortic narrowing is not hemodynamically significant. Short axis view of the aortic valve during diastole (#2) demonstrates an aortic bicuspid morphology (arrows). The right and left cusps are fused, resulting in the larger size of the anterior fused cusp (curved arrow); a small raphe (open arrow) is present. Bicuspid aortic valves are commonly associated with aortic coarctation; however, bicuspid aortic valves have also been described in the setting of pseudocoarctation. Coronal oblique MIP image (#3) shows dilation (open arrows) of the ascending aorta. \n\nComment: Bicuspid aortic valves are associated with ascending aortic aneurysms and dissection, even in the absence of significant aortic stenosis.', 'history': 'Patient with history of congential heart defect.', 'imagePoolId': '9aeb1d4b-6c4a-45a1-9416-30d6f8237358', 'name': 'Bicuspid aortic valve', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Coronal oblique graphic in a patient with pseudocoarctation of the aorta (PCOA) demonstrates an elongated, kinked, and buckled aortic arch distal to the origin of left subclavian artery at the level of the ductus arteriosus.*
+
+
+*Coronal oblique graphic in a patient with pseudocoarctation of the aorta (PCOA) demonstrates an elongated, kinked, and buckled aortic arch distal to the origin of left subclavian artery at the level of the ductus arteriosus.*
+
+
+*Sagittal oblique volume-rendered CTA shows kinking and mild narrowing of the proximal descending aorta at the level of the ligamentum arteriosum, consistent with pseudocoarctation. No enlarged collateral bronchial, intercostal, or internal mammary arteries are present.*
+
+
+*Posteroanterior chest radiograph shows a prominent and high-riding aortic arch . The normal heart size and absence of rib notching due to collateral vessels distinguish PCOA from coarctation of the aorta (COA).*
+
+
+*Lateral chest radiograph in the same patient demonstrates forward buckling of the aortic arch at the isthmus. The aorta is enlarged proximal to the narrowed segment .*
+
+
+*PA radiograph with bone subtraction (left) in a 67-year-old man shows abnormal elongation of the proximal descending thoracic aorta (DTA) , which can be visualized in coronal 3D volume-rendered image (right).*
+
+
+*Lateral radiograph shows elongation and superior extension of the aortic arch , which then courses inferiorly with kinking before taking its normal course . These findings are highly suggestive of pseudocoarctation.*
+
+
+*MIP (left) and volume-rendered (right) images in the same patient show aortic PCOA with elongation and superior extension of the proximal DTA , which then extends inferiorly with kinking before taking its normal course .*
+
+
+*PCOA in an asymptomatic 72-year-old woman shows elongation and kinking of the proximal DTA. There was no gradient across the lesion on phase-contrast MR. The aorta is aneurysmal distal to PCOA, measuring 3.5 cm.*
+
+
+*Sagittal 3D volume-rendered CTA in a patient with PCOA demonstrates kinking and mild narrowing of the proximal descending aorta at the level of the ligamentum arteriosum.*
+
+
+*Axial CECT in a patient with PCOA shows marked kinking and buckling of the aortic arch . No enlarged collateral bronchial, intercostal, or internal mammary arteries are identified.*
+
+
+### Additional Images
+
+
+*Axial PC MR obtained just distal to the pseudocoarctation shows an absence of increased flow velocity. Note that the bright signal in the ascending aorta indicates flow in the cephalad direction , and the dark signal in the descending aorta indicates caudal flow .*
+
+
+*Sagittal thin MIP contrast-enhanced MRA shows a very elongated and abnormal aortic arch with areas of aneurysmal dilatation of aortic arch proximal to and narrowing (PCOA). Note the abnormal origin of the left common carotid artery from the pseudocoarctation *
+
+
+*Coronal CTA shows a dilated and elongated right aortic arch , which is irregular and peripherally calcified. Note that there is extension of the aortic arch into the right lower neck , PCOA and presence of an aberrant left subclavian artery .*
+
+
+*Sagittal 3D volume-rendered CTA in the same patient shows an abnormally dilated and buckled aorta and the origins of the innominate and left subclavian arteries. (Courtesy S. Tanju, MD.)*
+
+
+*Coronal oblique thin MIP contrast-enhanced MRA shows aneurysmal dilatation of the aortic arch , which extends to the supraclavicular space and narrowing of distal aortic arch . Also note the stretching of the supraaortic arteries.*
+
+
+*Axial CTA in the same patient shows a dilated right aortic arch and a high-grade stenosis of the origin of the aberrant left subclavian artery . (Courtesy S. Tanju, MD.)*
+
+
+*Sagittal reformatted black-blood MR shows narrowing of the thoracic aorta distal to the left subclavian artery . Phase-contrast flow quantification (not shown) revealed normal peak velocity and gradient across the narrowed segment, indicating PCOA.*
+
+
+*Axial black-blood MR demonstrates size discrepancy between the ascending aorta and the proximal DTA at isthmus . Note the lack of collateral vessels consistent with PCOA.*
+
+
+*Sagittal reformatted CECT shows kinking of distal aortic arch with narrowing of the aortic isthmus just distal to the left subclavian artery . Note severe aortic valve calcifications in this patient with associated aortic valve stenosis .Abnormalities commonly associated with PCOA include aortic stenosis, bicuspid aortic valve, and left-to-right shunts.*
+
+
+*3D volume-rendered CTA in a patient with PCOA shows narrowing of the aortic arch and dilatation of the aorta proximal to the narrowed segment . Note the diminished size of the DTA distal to the site of narrowing.*
+
+
+*3D volume-rendered CTA in the same patient demonstrates marked kinking and tortuosity of the aortic arch . The lack of poststenotic dilatation and absence of collateral vessels are consistent with PCOA.*
+
+
+*Axial CECT in a patient with PCOA demonstrates kinking and buckling of the aortic arch, which is elongated and tortuous . Note the notch in the distal transverse aortic arch at the attachment of the ligamentum arteriosum.*
+
+
+*Axial CECT in a patient with PCOA shows dilatation of the aorta proximal to the narrowed segment and diminished size of the DTA distal to the narrowed segment . Note the lack of collateral vessels in the mediastinum.*
+
diff --git a/docs_md/articles/right-aortic-arch_5f186c96-4cc3-453e-840d-12ebfad13115.md b/docs_md/articles/right-aortic-arch_5f186c96-4cc3-453e-840d-12ebfad13115.md
new file mode 100644
index 0000000..9aa7d33
--- /dev/null
+++ b/docs_md/articles/right-aortic-arch_5f186c96-4cc3-453e-840d-12ebfad13115.md
@@ -0,0 +1,442 @@
+---
+title: "Right Aortic Arch"
+docid: "5f186c96-4cc3-453e-840d-12ebfad13115"
+authors:
+ - key: "ee6ece9d-ad74-458c-a8df-11628ae7f879"
+ value: "Arzu Canan, MD"
+ - key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
+ value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Right Aortic Arch"
+ slug: "right-aortic-arch"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "eaaa45f9-6ee4-4b8f-aa58-f6293c1ca1d5"
+imageCount: 27
+lastUpdated: "01/24/25"
+pageDescription: "Right Aortic Arch"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Right Aortic Arch"
+pageTitle: "Right Aortic Arch | STATdx"
+enhancedTitle: "Right Aortic Arch"
+type: "DX"
+references: true
+anatomy:
+ - "{'authors': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI; Seth Kligerman, MD, MS', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/e4b09a25-666c-486b-b040-a9af4ae560b4', 'category': 'Cardiac', 'compareUrl': '/compare/document/e4b09a25-666c-486b-b040-a9af4ae560b4/related-anatomy/treeNode?subContext=Coronary Anatomy', 'documentId': 'e4b09a25-666c-486b-b040-a9af4ae560b4', 'documentType': 'ANATOMY', 'documentUrl': '/document/coronary-anatomy/e4b09a25-666c-486b-b040-a9af4ae560b4', 'enhancedTitle': 'Coronary Anatomy', 'entryDate': '01/27/25', 'imageCount': 26, 'imageUrl': '/image/thumbnail/54751af7-dd29-4279-bf86-8e803c85351c?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 4, 'showCompareButton': False, 'title': 'Coronary Anatomy'}"
+cases: 2
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Right Aortic Arch"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Right aortic arch (RAA)
+ - Aortic arch located to right of trachea
+ - Common variations
+ - RAA with aberrant left subclavian artery (ALSA) ± Kommerell diverticulum (KD)
+ - RAA with mirror-image branching
+- ## Imaging
+
+
+ - Radiography
+ - Right paratracheal nodular opacity and indentation of right tracheal margin on frontal chest radiograph
+ - KD: Retroesophageal opacity with indentation of posterior tracheal margin on lateral chest radiograph
+ - CT
+ - RAA with ALSA with retroesophageal course ± KD
+ - RAA with mirror-image branching
+ - RAA with left descending aorta with retroesophageal aortic segment
+- ## Top Differential Diagnoses
+
+
+ - Double aortic arch
+ - Mediastinal mass
+- ## Clinical Issues
+
+
+ - RAA with ALSA
+ - Most patients are asymptomatic
+ - Some patients with KD may have dysphagia or stridor
+ - RAA with mirror-image branching
+ - Cyanotic congenital heart disease
+ - RAA with left descending aorta (circumflex aorta)
+ - Ductus ligament between pulmonary artery and ALSA constitutes vascular ring
+ - Treatment
+ - Symptomatic RAA with ALSA/KD may require division of ligamentum via left thoracotomy
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Right aortic arch (RAA)
+- ## Synonyms
+
+
+ - Right arch
+- ## Definitions
+
+
+ - Aortic arch located to right of trachea, crossing right main stem bronchus
+ - Common variations
+ - RAA with mirror-image branching
+ - RAA with aberrant left subclavian artery (ALSA) ± Kommerell diverticulum (KD)
+ - KD
+ - Saccular dilatation at level of ALSA
+ - Implies left-sided ligamentum arteriosum and vascular ring
+ - Uncommon variations
+ - RAA with left descending aorta (circumflex aorta)
+ - RAA with isolation of left subclavian artery
+ - RAA with aberrant brachiocephalic artery
+ - RAA with unilateral absence of pulmonary artery
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Indentation of right tracheal margin due to paratracheal mass
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - General features for different variations
+ - Right paratracheal opacity
+ - Indentation of right tracheal margin
+ - Normally, left aortic arch (LAA) has indentation along left tracheal margin
+ - RAA with ALSA
+ - + KD
+ - Retroesophageal nodular opacity
+ - Indentation of posterior tracheal margin
+ - Can simulate left aortic arch (LAA) on frontal projection
+ - RAA with mirror-image branching
+ - ± dextrocardia
+ - High association with congenital heart disease
+ - Tetralogy of Fallot
+ - Truncus arteriosus
+ - Tricuspid atresia
+ - Transposition of great arteries
+ - RAA with unilateral absence of pulmonary artery
+ - Hypoplastic ipsilateral hemithorax with contralateral hyperinflation
+ - Absent or grossly ↓ pulmonary vascular markings
+- ## Fluoroscopic Findings
+
+
+ - ### Esophagram
+
+
+ - Indentations seen only when ALSA present
+ - Frontal projection: Oblique filling defect coursing from right inferior to left superior
+ - Lateral view: Posterior indentation
+ - Large posterior indentation: Aortic diverticulum
+- ## CT Findings
+
+
+ - RAA with ALSA (most common)
+ - 4 great vessels from left to right in following order: Left common carotid, right common carotid, right subclavian, ALSA
+ - ALSA with retroesophageal course
+ - ± KD (bulbous dilatation at origin of ALSA)
+ - RAA with mirror-image branching
+ - 3 great vessels from left to right in following order: Left brachiocephalic, right common carotid, right subclavian
+ - Ancillary findings of congenital heart disease (e.g., tetralogy of Fallot and truncus arteriosus)
+ - Rarely, blind aortic diverticulum (similar to KD)
+ - Difficult to differentiate from double aortic arch (DAA) with atretic left arch
+ - Inferior tethering of left subclavian artery may suggest DAA with atretic left arch
+ - Both RAA with mirror-image branch and aortic diverticulum and DAA with atretic left arch are vascular rings
+ - RAA with left descending aorta (circumflex aorta)
+ - Retroesophageal aortic segment
+ - Variants
+ - Mirror-image branching
+ - ALSA
+ - Left descending thoracic aorta
+ - RAA with isolation of left subclavian artery
+ - 3 great vessels from left to right in following order: Left carotid, right carotid, right subclavian
+ - Left subclavian artery is blind ended, connected to aortic arch by ductus ligament
+ - RAA with aberrant brachiocephalic artery
+ - Retroesophageal course of left carotid and left subclavian arteries
+ - RAA with unilateral absence of pulmonary artery
+ - RAA with ALSA or mirror-image branching
+ - Pulmonary artery terminates within 1 cm of its origin
+ - More peripheral pulmonary arteries are present
+ - Pulmonary hypertension
+ - Ancillary findings: Bronchiectasis, mosaic attenuation, and honeycombing
+- ## MR Findings
+
+
+ - Same accuracy as CT to assess for variations of vascular anatomy
+ - Absence of ionizing radiation is significant advantage, especially in younger patients
+ - MR superior to CT in setting of complex congenital heart disease
+ - Can be evaluated with contrast or noncontrast MR angiography
+- ## Echocardiographic Findings
+
+
+ - ### Echocardiogram
+
+
+ - May determine great vessels pattern (mirror-image branching vs. ALSA), although there are acoustic window limitations
+ - Origin of ALSA usually well seen, especially in presence of KD
+ - Helpful in further characterizing associated congenital heart disease
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - Characterization of different variations of RAA successfully achieved with either MR or CT
+ - MR superior in fully characterizing congenital heart disease in setting of RAA with mirror-image branching
+
+# DIFFERENTIAL DIAGNOSIS
+
+- [Double Aortic Arch](/document/double-aortic-arch/0d855dcf-4165-419c-8d94-c59d81e27fb8)
+ - Differentiation on radiography may be not possible, as KD can simulate LAA
+ - CT and MR are diagnostic
+ - Patent RAA and LAA with larger RAA and smaller LAA
+ - Double aortic arch (DAA) with atretic LAA
+ - Inferior tethering of left subclavian artery
+ - Aortic diverticulum more common in DAA
+- ## Mediastinal Mass
+
+
+ - Right paratracheal lymphadenopathy and esophageal neoplasm can simulate RAA on chest radiography
+ - Focal and smooth indentation of right tracheal margin classically seen in RAA on chest radiography
+ - CT or MR better delineate mediastinal masses and differentiate from RAA
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Congenital derangement
+ - Embryologic considerations
+ - Causes of arch anomalies
+ - Interruption of normal parts
+ - Lack of interruption of parts that normally regress
+ - LAA (normal aortic arch) develops from interruption distal to right subclavian artery
+ - RAA with ALSA develops from interruption between left common carotid and left subclavian arteries
+ - RAA with mirror-image branching develops from interruption distal to left subclavian artery
+ - ### Associated abnormalities
+
+
+ - RAA with ALSA ± KD: Low incidence of congenital heart disease
+ - RAA with mirror-image branching: High incidence of congenital heart disease (~ 98%)
+ - RAA in 25% of patients with tetralogy of Fallot
+ - RAA in 25-50% of patients with truncus arteriosus
+ - Transposition of great vessels
+ - RAA rarely associated with unilateral absence of pulmonary artery
+- ## Gross Pathologic & Surgical Features
+
+
+ - KD
+ - Remnant of embryonic left 4th aortic arch
+ - Implies ipsilateral (left) ductus ligament and resulting vascular ring (often loose)
+ - Embryologically different from ALSA
+ - ALSA
+ - Can arise directly from descending aorta or from DK
+ - Rarely lies anterior to trachea (5%)
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - RAA with ALSA
+ - Most patients asymptomatic
+ - Presence of KD constitutes vascular ring, often loose
+ - Patients with KD may have dysphagia or stridor
+ - RAA with mirror-image branching
+ - Cyanotic congenital heart disease
+ - If associated with blind aortic diverticulum (but no ALSA), constitutes loose vascular ring that may be symptomatic (i.e., dysphagia, stridor)
+ - RAA with left descending aorta (a.k.a. circumflex aorta)
+ - Ductus ligament between pulmonary artery and ALSA constitutes vascular ring, often loose, that may be symptomatic (i.e., dysphagia, stridor)
+- ## Demographics
+
+
+ - ### Age
+
+
+ - The tighter the vascular ring, the earlier it becomes symptomatic
+ - ### Epidemiology
+
+
+ - RAA present in 0.1% of adults
+ - RAA with ALSA is most common variant
+- ## Natural History & Prognosis
+
+
+ - Determined mostly by coexisting congenital heart disease
+ - Tracheomalacia, residual stenosis, and vascular compression are common after repair, which most children eventually outgrow, but some require additional surgery
+ - RAA with ALSA: Association with progressive stenosis of ALSA has been described in pediatric population
+ - RAA with mirror-image branching: High association with stenosis of common carotid arteries or subclavian arteries
+- ## Treatment
+
+
+ - Symptomatic RAA with ALSA/KD
+ - Requires division of ligamentum via left thoracotomy
+ - Aortopexy may be needed additionally
+ - When associated with complete cartilaginous tracheal ring: Resection and tracheal reconstruction
+ - RAA with mirror-image branching
+ - Treatment of associated congenital heart disease
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - RAA with mirror-image branching pattern must be evaluated for congenital heart disease
+ - RAA with ALSA but no KD is usually incidental finding (i.e., asymptomatic)
+ - RAA with ALSA and KD constitutes vascular ring
+ - While frequently asymptomatic, few patients may present with dysphagia &/or stridor
+- ## Image Interpretation Pearls
+
+
+ - RAA variant may be impossible to establish on chest radiography
+ - MR and CT equally accurate to determine pattern of branching in patients with RAA
+
+ c4b1211f-7ef2-4e83-8e0d-6ad126698010
+
+## References
+
+# Selected References
+
+1. [Prabhu S et al: Anatomic classification of the right aortic arch. Cardiol Young. 30(11):1694-701, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33109287%5Bpmid%5D)
+1. [Muraoka M et al: High incidence of progressive stenosis in aberrant left subclavian artery with right aortic arch. Heart Vessels. 33(3):309-15, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28965152%5Bpmid%5D)
+1. [Arazińska A et al: Right aortic arch analysis: anatomical variant or serious vascular defect? BMC Cardiovasc Disord. 17(1):102, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28420337%5Bpmid%5D)
+1. [Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27860551%5Bpmid%5D)
+1. [Tanaka A et al: Kommerell's diverticulum in the current era: a comprehensive review. Gen Thorac Cardiovasc Surg. 63(5):245-59, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25636900%5Bpmid%5D)
+1. [Etesami M et al: Computed tomography in the evaluation of vascular rings and slings. Insights Imaging. 5(4):507-21, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25008430%5Bpmid%5D)
+1. [Kanne JP et al: Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 4(5):293-300, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20829147%5Bpmid%5D)
+1. [Türkvatan A et al: Congenital anomalies of the aortic arch: evaluation with the use of multidetector computed tomography. Korean J Radiol. 10(2):176-84, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19270864%5Bpmid%5D)
+1. [Holmes KW et al: Magnetic resonance imaging of a distorted left subclavian artery course: an important clue to an unusual type of double aortic arch. Pediatr Cardiol. 27(3):316-20, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16565909%5Bpmid%5D)
+1. [Weinberg PM: Aortic arch anomalies. J Cardiovasc Magn Reson. 8(4):633-43, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16869315%5Bpmid%5D)
+1. [Knight L et al: Right aortic arch. Types and associated cardiac anomalies. Circulation. 50(5):1047-51, 1974](http://www.ncbi.nlm.nih.gov/pubmed/?term=4430090%5Bpmid%5D)
+
+## Anatomy
+
+### Coronary Anatomy
+Cardiac/ANATOMY:e4b09a25-666c-486b-b040-a9af4ae560b4
+
+## Cases
+
+- {'cases': [{'authors': [{'key': 'b00d2bdb-66e1-41ed-90b4-c52904f4d598', 'value': 'Seth Kligerman, MD, MS'}], 'caseVersionId': '203a2db9-3625-4686-8399-cf8c28252385', 'description': 'PA radiograph (#1) shows a right-sided aortic arch (arrow), which indents the right side of the trachea (curved arrow). Notice that the descending thoracic aorta is to the right of the spine (open arrow). Lateral radiograph (#2) shows no evidence of a retroesophageal density to suggest an aberrant left subclavian artery. Also notice the small median sternotomy wires from prior cardiac surgery when the patient was an infant (arrow). Axial CT image at the level of the arch (#3) confirms the presence of a right-sided aortic arch (arrow). No aberrant left subclavian artery is present. Axial image above the arch (#4) shows 4 vessels originating from the superior aspect of the aortic arch. These are the right subclavian artery (black arrow), right common carotid artery (curved arrow), left common carotid artery (open arrow), and left subclavian artery (white arrow). This orientation with absence of an aberrant left subclavian artery confirms mirror image branching. Coronal maximum intensity projection reformat (#5) shows the right subclavian artery (black arrows), right common carotid artery (curved arrow), left common carotid artery (open arrow), and left subclavian artery (white arrow) arising from the superior aspect of the aortic arch. The origin of the right subclavian artery is partially obscured by contrast in the superior vena cava. Coronal maximum intensity projection reformat (#6) shows the right-sided descending thoracic aorta (arrow). Axial CT image (#7) shows right ventricular dilatation and a right-sided descending thoracic aorta (arrow), both associated with tetralogy of Fallot.', 'history': 'Patient who underwent surgical repair for tetralogy of Fallot when 7 months old. ', 'imagePoolId': 'f9e793dc-db2d-4b00-97d2-a507aa1ca9dd', 'name': 'Right arch, mirror image branching, tetralogy', 'teachingPoint': None, 'demographics': '14 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '4653106c-5a00-4f0a-95e0-9261e606fca8', 'description': 'Typical radiographic and CT features of bronchiectasis from immotile cilia syndrome.\n\nFrontal radiograph (#1) shows situs inversus: right aortic arch (arrow) and dextrocardia (curved arrow). Hiatal hernia (open arrow). Lateral radiograph (#2) shows hiatal hernia (arrow) and possible bronchiectasis (open arrows). NECT (#3-5) shows diffuse bronchiectasis (open arrows).', 'history': 'Chronic cough.', 'imagePoolId': '275d846a-6dd9-41f4-a759-81e5cd2a01fc', 'name': 'Bronchiectasis', 'teachingPoint': None, 'demographics': '59 Years old female'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '48579a91-0ef2-4c4b-8c1d-cdbc611e860b', 'description': 'Typical radiographic and CT features of bronchiectasis from immotile cilia syndrome.\n\nRadiograph (#1) shows situs inversus: right aortic arch (curved arrow), dextrocardia (open arrow), right-sided gastric bubble (black arrow) and bronchiectasis left lower lobe (open arrow). Lateral radiograph (#2) shows left middle lobe atelectasis (arrows). NECT (#3) shows bronchiectasis and left middle lobe atelectasis (white arrow). Right-sided empyema (black arrows). NECT (#4) shows situs inversus. Radiograph different time (#5) shows left lower lobe bronchiectasis (arrow).', 'history': 'Chronic cough.', 'imagePoolId': '6083a376-687a-423c-bf5f-12fbdc5c47b3', 'name': 'Bronchiectasis', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '55c0cff5-49cc-4f10-af0f-9bbe4379c141', 'value': 'Howard Mann, MD'}], 'caseVersionId': '4c3ff2ad-5bc1-4d91-b0b3-b15ae55af634', 'description': 'Typical radiographic features of right aortic arch.\n\nRadiographs (#1, 2) demonstrate abnormal course of the aortic arch as well as descending aorta (arrows). Note the absence of aortic knob in normal position (curved arrow, #1).', 'history': 'Incidental finding. ', 'imagePoolId': '5fd06a4d-700c-44f5-bba6-25e9ad8f483b', 'name': 'Right paratracheal mass', 'teachingPoint': None}, {'authors': [{'key': 'fff9b5a0-8473-401a-8da6-d1366705ec01', 'value': 'Jeffrey P. Kanne, MD'}], 'caseVersionId': '5cc33a02-bc3e-4cdb-ac1d-dc3a521f8bc1', 'description': "PA radiograph (#1) shows a well-defined mediastinal mass (black arrows) displacing the trachea to the left. No aortic arch is apparent on the left. The descending aorta (open black arrows) can be seen coursing along the right of the mediastinum, crossing to left just above the diaphragm, and entering the abdomen. Lateral radiograph (#2) shows a well-defined round mass (black arrows) in the retrotracheal (Raider's) triangle displacing the trachea anteriorly.\n\nAxial CECT (#3) and coronal thin-slab CT maximum-intensity projection (#4) show a right aortic arch (white arrows) with an aberrant left subclavian artery (open white arrow, #3) arising from a large diverticulum of Kommerell (curved white arrow, #3).", 'history': 'Acute chest pain.', 'imagePoolId': 'a10a28d7-2530-4292-adce-9d33dd60cc1b', 'name': 'Right aortic arch with aberrant left subclavian artery', 'teachingPoint': None, 'demographics': '49 Years old male'}, {'authors': [{'key': '924986ad-eb92-480e-88ae-1f3d01d70763', 'value': 'James G. Ravenel, MD'}], 'caseVersionId': 'ea283083-a2d4-4b44-b794-34cce427948f', 'description': 'Typical radiographic features of right paratracheal mass due to right aortic arch.\n\nRadiographs (#1,2) show right aortic arch (arrows) deviating the trachea to the left. On lateral view (#2), aberrant left subclavian courses posterior to trachea, bowing posterior wall anteriorly (curved arrow).', 'history': 'Pre-operative examination', 'imagePoolId': '68d80a8d-e2fe-414b-ba19-f2ca6b23d271', 'name': 'Right paratracheal mass', 'teachingPoint': None, 'demographics': '70 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'ea90705e-d115-452d-9950-d569bd302c08', 'description': 'Typical radiographic features of right paratracheal mass from right aortic arch.\n\nRadiographs (#1,2) show right aortic arch (arrow) shifting the trachea to the left. Absence of normal aortic mogul on the left (open arrow). Lateral radiograph (#3) is normal.', 'history': 'Asymptomatic.', 'imagePoolId': '5b3fafeb-9ecd-476c-bae3-724541e6a07a', 'name': 'Right paratracheal mass', 'teachingPoint': None, 'demographics': '34 Years old female'}, {'authors': [{'key': 'b00d2bdb-66e1-41ed-90b4-c52904f4d598', 'value': 'Seth Kligerman, MD, MS'}], 'caseVersionId': '8fa27665-18fc-48f7-a70e-a2103c305859', 'description': "PA radiograph (#1) shows a right-sided aortic arch (black arrow), which is indenting on the right wall of the trachea (open arrow). Also notice the prominent vascular structure to the left of the trachea at the same level (curved arrow). Although this structure appears contiguous with the aorta, it does not indent the left wall of the trachea, suggesting that it is either anterior or posterior to the trachea. The thoracic aorta is tortuous and descends to the right of the spine (white arrow). Lateral radiograph (#2) shows a large vascular structure displacing the trachea anteriorly (arrow). This structure appears contiguous with the shadow of the descending thoracic aorta (open arrow). \n\nAxial CT images at the level of the ascending aorta (#3-4) show a right-sided aortic arch (arrows), which displace the trachea to the left. Additionally, there is a large diverticulum of Kommerell (black open arrows) coursing posterior to both the trachea and esophagus (curved arrows), displacing both structures anteriorly. This diverticulum of Kommerell gives rise to the left subclavian artery (white open arrows). This constellation of findings represents a right aortic arch with an aberrant left subclavian artery. Incidentally, the origin of the left subclavian artery was stenotic due to atherosclerotic disease. Axial CT image more inferior (#5) shows the right-sided descending thoracic aorta (arrow). Also notice the esophageal stent and the markedly thickened esophagus (open arrow) due to the patient's esophageal cancer. Sagittal CT reformat (#6) shows the large diverticulum of Kommerell (arrow) coursing posterior to the trachea and esophagus, displacing both structures anteriorly. Coronal reformat (#7) demonstrates aortic morphology with the right-sided aortic arch (arrow) and the large diverticulum of Kommerell (open arrow), which gives rise to the left subclavian artery (curved arrow).", 'history': 'Patient with history of esophageal cancer undergoes yearly CT examination to evaluate for disease recurrence. ', 'imagePoolId': '312d0e10-4e9a-4279-858a-53ffa0ad43b0', 'name': 'Aberrant left subclavian', 'teachingPoint': None, 'demographics': '64 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': 'a5d3a4eb-860a-429a-b9a9-6897de9309c2', 'value': ' HELIMED, Katowice, Poland'}], 'caseVersionId': 'a7b35684-4e2b-4f58-93b9-0cccdde60f9b', 'description': 'Typical radiographic and CT features of right aortic arch.\n\nAP localizer (#1) show right aortic arch (arrows). Normal shadow of aortic knob is absent (curved arrow). CT image (#2) shows right aortic arch (arrow). CT images (#3-5) show the course of the descending aorta on the right (arrows).', 'history': 'Incidental finding. ', 'imagePoolId': '14b7f023-a2af-4694-ae96-14a5bb196304', 'name': 'Right paratracheal mass', 'teachingPoint': None, 'demographics': '70 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '7062283f-41be-4456-80ab-d1b33a559bc1', 'description': 'Variant CT features of right aortic arch, aberrant left subclavian artery, obstructing the esophagus causing dysphagia lusoria.\n\nCECT (#1-7) shows right aortic arch (arrows, #2-7), aberrant left subclavian artery (open arrows, #1-4), and dilated esophagus (curved arrow, #1,2).', 'history': 'Dysphagia lusoria.', 'imagePoolId': '06362dc6-87e3-44f6-8ad8-de64b798f2ae', 'name': 'Dilated esophagus', 'teachingPoint': None, 'demographics': '48 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'e4bc6099-3a98-416d-8ecc-c33685ee8894', 'description': 'Variant right cervical arch and vascular ring causing tracheomalacia.\n\nRadiographs (#1-4) shows right cervical aortic arch (arrows). CECT (#5, 6) shows right cervical aortic arch (arrow), aberrant left subclavian artery (open arrow), and tracheal compression (curved arrows). Sagittal reconstruction (#7) shows tracheal compression by aberrant left subclavian artery (arrow). Three-dimensional reconstruction of the trachea (#8) shows posterior narrowing (arrow). Three-dimensional reconstruction (#9) shows aberrant left subclavian (open arrows) and ductus ligament (arrow). \n\nComment: Dilated origin of aberrant subclavian known as diverticulum of Kommerell. In the case, with cervical arch vascular structures crowded into narrow inlet causing compression and tracheomalacia.', 'history': 'Dyspnea on exertion, wheezing, "asthma."', 'imagePoolId': 'ce528799-4648-472e-ab95-075b09653bf4', 'name': 'Cervical arch', 'teachingPoint': None, 'demographics': '24 Years old female'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*PA chest radiograph in a patient with right aortic arch (RAA) and aberrant left subclavian artery (ALSA) without Kommerell diverticulum (KD) shows RAA as a right paratracheal nodular opacity with right tracheal indentation.*
+
+
+*PA chest radiograph in a patient with right aortic arch (RAA) and aberrant left subclavian artery (ALSA) without Kommerell diverticulum (KD) shows RAA as a right paratracheal nodular opacity with right tracheal indentation.*
+
+
+*Lateral chest radiograph in the same patient shows the normal configuration of the trachea . ALSA with KD can show indentation of posterior tracheal margin; however, differentiation from ALSA without KD is not always possible.*
+
+
+*Axial chest CTA in an asymptomatic patient with RAA , ALSA , and descending thoracic aorta on the right reveals an incidentally noted persistent left superior vena cava draining into the coronary sinus.*
+
+
+*Coronal reformation CTA in the same patient shows ALSA arising as the last aortic branch. There is no KD. The lack of KD usually indicates absence of a ductus ligament on the side of the anomalous subclavian artery (SCA); therefore, this does not constitute a vascular ring.*
+
+
+*PA chest radiograph in an asymptomatic patient with RAA, ALSA, and KD shows RAA as a right paratracheal nodular opacity with an indentation of the right tracheal margin.*
+
+
+*Lateral chest radiograph in the same patient shows an indentation of the posterior tracheal margin , which relates to the presence of a KD. This suggests that mirror-image branching is not present. Note that KD implies the presence of a vascular ring, which may or may not be symptomatic.*
+
+
+*Composite axial chest CTA in a patient with RAA, ALSA, and KD demonstrates RAA and ALSA/KD with mild dilatation of the proximal esophagus superior to the KD.*
+
+
+*Sagittal chest CTA reformation in the same patient shows posterior tracheal indentation by the KD . KD implies the presence of a ductus ligament contralateral to the arch and, hence, constitutes a vascular ring. KD predisposes to dysphagia when present (dysphagia lusoria).*
+
+
+*Oblique coronal chest CTA reformation in the same patient shows a bulbous configuration of the origin of the ALSA, a classic feature of KD. KD is a consequence of the presence of a ductus arteriosus on the side of the SCA. Pathophysiologically, patients with KD may have symptoms (e.g., dysphagia lusoria) related to a vascular ring completed by the ligamentum arteriosum.*
+
+
+*Anterior and posterior CTA 3D reformations in the same patient with RAA, ALSA , and KD show the bulbous appearance of the KD.*
+
+
+*Composite axial chest CTA shows RAA with a retroesophageal left descending aorta (circumflex aorta) , blind aortic diverticulum , and mirror-image branching.*
+
+
+*Composite chest CTA coronal reformation in the same patient shows RAA , left brachiocephalic trunk , blind aortic diverticulum , and left descending aorta . The circumflex aorta implies a vascular ring, which often is loose and occasionally symptomatic.*
+
+
+*Axial chest CTA in an infant with RAA with mirror-image branching and aortic diverticulum with stridor shows a right-sided descending thoracic aorta .*
+
+
+*Posterior 3D CTA in the same patient is shown. Despite the presence of a blind aortic diverticulum , this case represents an RAA, not a double aortic arch (DAA) with atretic left aortic arch (LAA), given the lack of inferior tethering of the left SCA. (Courtesy R. Reina, MD.)*
+
+
+*Axial chest CECT shows RAA with isolation of the SCA. Note the presence of prominent arterial collaterals .*
+
+
+*Coronal chest CECT in the same patient shows the blind origin of the left SCA with a cord-like structure extending from it to the aortic wall representing a ductus ligament , which can clinically represent a loose ring. Note extensive arterial collaterals , which supply the left SCA.*
+
+
+### Additional Images
+
+
+*PA chest radiograph in a patient with RAA , ALSA, and KD is shown. Frequently, when an RAA is associated with a KD, the latter can sometimes simulate the presence of a normal LAA, thus appearing as a DAA on frontal chest radiograph. An important clue for differentiation is that the KD does not exert an indentation on the left lateral margin of the trachea, as would be expected with a coexistent LAA.*
+
+
+*Frontal DSA aortogram in a patient with RAA also shows ALSA and KD .*
+
+
+*Coronal chest CTA reformation in a patient with RAA, ALSA, and KD shows mild normal indentation of the right tracheal margin from the RAA. In this case, there is no significant stenosis of the trachea.*
+
+
+*Frontal projection esophagram in a pediatric patient with RAA and ALSA shows an oblique indentation on the esophageal lumen, which is caused by the ALSA. Note that esophagrams are often useful when assessing vascular rings in symptomatic patients.*
+
+
+*Axial CTA shows RAA with mirror-image branching in a patient with tetralogy of Fallot.*
+
+
+*Radiograph shows a leftward position of the trachea in a patient who is rotated to the left.*
+
+
+*Coronal rendition in the same patient shows a takeoff of the left SCA from the KD.*
+
+
+*Changing the opacity setting of this rendition reveals the presence of an ALSA .*
+
+
+*Axial CTA in an asymptomatic infant with stridor shows an RAA and ALSA . Note mild tracheal narrowing and also bilateral superior venae cavae.*
+
+
+*Coronal CTA volume rendition shows an RAA with no tracheal compression.*
+
+
+*Axial CTA in an asymptomatic 49-year-old woman shows an RAA and aneurysmally dilated KD .*
+
diff --git a/docs_md/articles/takayasu-arteritis_d35eb6f3-bfd3-4121-8781-325a93ccc197.md b/docs_md/articles/takayasu-arteritis_d35eb6f3-bfd3-4121-8781-325a93ccc197.md
new file mode 100644
index 0000000..58370df
--- /dev/null
+++ b/docs_md/articles/takayasu-arteritis_d35eb6f3-bfd3-4121-8781-325a93ccc197.md
@@ -0,0 +1,443 @@
+---
+title: "Takayasu Arteritis"
+docid: "d35eb6f3-bfd3-4121-8781-325a93ccc197"
+authors:
+ - key: "ee6ece9d-ad74-458c-a8df-11628ae7f879"
+ value: "Arzu Canan, MD"
+ - key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
+ value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Takayasu Arteritis"
+ slug: "takayasu-arteritis"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "1f530233-4bd8-423a-9dcc-253d9f03082a"
+imageCount: 12
+lastUpdated: "12/19/24"
+pageDescription: "Takayasu Arteritis"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Takayasu Arteritis"
+pageTitle: "Takayasu Arteritis | STATdx"
+enhancedTitle: "Takayasu Arteritis"
+type: "DX"
+references: true
+tables: 1
+anatomy:
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+ - "{'authors': 'Anne G. Osborn, MD, FACR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'category': 'Brain', 'compareUrl': '/compare/document/a7a252f0-2ac6-402a-8c87-cfce8adc799b/related-anatomy/treeNode?subContext=Aortic Arch and Great Vessels', 'documentId': 'a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'documentType': 'ANATOMY', 'documentUrl': '/document/aortic-arch-and-great-vessels/a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'enhancedTitle': 'Aortic Arch and Great Vessels', 'entryDate': '10/20/20', 'imageCount': 8, 'imageUrl': '/image/thumbnail/5a451d53-7fe4-426e-b816-7f0e0a1df745?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Aortic Arch and Great Vessels'}"
+ - "{'authors': 'Melissa L. Rosado-de-Christenson, MD, FACR, FAAWR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/551f4b47-fac1-44f9-b800-09ce766fedd9', 'category': 'Chest', 'compareUrl': '/compare/document/551f4b47-fac1-44f9-b800-09ce766fedd9/related-anatomy/treeNode?subContext=Pulmonary Vessels', 'documentId': '551f4b47-fac1-44f9-b800-09ce766fedd9', 'documentType': 'ANATOMY', 'documentUrl': '/document/pulmonary-vessels/551f4b47-fac1-44f9-b800-09ce766fedd9', 'enhancedTitle': 'Pulmonary Vessels', 'entryDate': '10/10/23', 'imageCount': 90, 'imageUrl': '/image/thumbnail/ce1325b9-6730-40c4-992d-7eaec8c5ff4f?size=174&quality=85', 'inCompareCart': False, 'rank': 3, 'referenceCount': 8, 'showCompareButton': False, 'title': 'Pulmonary Vessels'}"
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+cases: 1
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Takayasu Arteritis"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Takayasu arteritis (TA)
+ - Pulseless disease
+ - Chronic granulomatous vasculitis of large vessels
+- ## Imaging
+
+
+ - Best diagnostic clue: Wall thickening of large vessels
+ - Thoracic aorta and branches
+ - Pulmonary artery involvement is less common
+ - NECT: Aortic wall thickening
+ - CECT: Aortic wall thickening and enhancement
+ - Stenosis, occlusion, aneurysm
+ - MRA: Aortic narrowing, dilatation
+ - Angiography: 4 types classified by location
+ - PET/CT is used for treatment monitoring
+ - Complications
+ - Stenosis > occlusion
+ - Aneurysm
+ - Dissection
+- ## Top Differential Diagnoses
+
+
+ - Giant cell arteritis
+ - Aortic coarctation
+- ## Pathology
+
+
+ - Autoimmune etiology is suspected
+ - Specific types of human leukocyte antigen are common among patients
+- ## Clinical Issues
+
+
+ - Disease stages
+ - Early or prepulseless phase
+ - Vascular inflammatory phase
+ - Late quiescent occlusive or pulseless phase
+ - Triphasic disease in minority of patients
+ - F:M = 8:1
+ - Heart failure is most common cause of death
+ - Treatment
+ - Corticosteroids, angioplasty, surgical bypass
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - **Takayasu ar****teritis (TA)**
+- ## Synonyms
+
+
+ - **Pulseless disease**
+- ## Definitions
+
+
+ - Chronic granulomatous vasculitis of large vessels
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - Wall thickening of large vessels
+ - ### Location
+
+
+ - **Thoracic aorta****and****branches**
+ - **Left subclavian artery** is most commonly affected
+ - Ostial stenoses or occlusion of arch vessels
+ - Pulmonary artery (PA) involvement is less common
+ - Evidence of medium or large vessel involvement on imaging is absolute requirement for diagnosis of TA
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - Irregular or dilated descending thoracic aorta
+ - Diminished pulmonary vessels and rib notching
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - Vessel wall thickening, iso-/hyperdense to muscle
+ - ### CECT
+
+
+ - **Vessel wall thickening****and****enhancement**
+ - **Stenosis, occlusion, aneurysm**
+ - 95% sensitivity and 100% specificity for diagnosis of TA
+ - Delayed/venous phase: Double ring enhancement pattern
+ - Hyperenhanced outside ring: Active inflammation of media and adventitia
+ - Poor enhancing inside ring: Swelling of intima
+ - Can be used to assess treatment response
+- ## MR Findings
+
+
+ - ### T1WI
+
+
+ - **Wall thickening: Aorta****and****branches**
+ - Wall edema can be seen in STIR images
+ - ### T1WI C+
+
+
+ - **Enhancement of thickened vessel wall**
+ - ### MRA
+
+
+ - Focal/diffuse narrowing of aorta and branches
+ - Aortic dilatation (ascending > descending)
+ - Stenosis > occlusion
+ - Aortic regurgitation, dissection, aneurysm
+ - Disease activity
+ - Active disease: Wall edema and delayed enhancement can be seen
+ - Poor and inconsistent correlation with disease activity
+ - Development of new lesion in previously unaffected vascular territory is evidence of active disease/progression
+ - Evaluate for new lesions in other territories
+- ## Ultrasonographic Findings
+
+
+ - ### Grayscale ultrasound
+
+
+ - Vascular wall thickening, dilation, stenosis
+ - Diffuse arterial wall thickening: Macaroni sign
+ - Carotid intima-media thickness > 1 mm is considered to be inflammatory vascular disease
+ - ### Color Doppler
+
+
+ - Luminal stenosis, change in blood flow patterns
+ - 81% sensitivity and 100% specificity for diagnosing TA
+- ## Angiographic Findings
+
+
+ - Early: Aortic wall thickening, rarely, stenosis
+ - Late: Stenosis, occlusion, aneurysm; 4 types
+ - I: Aortic arch branches
+ - II: Thoracic aorta (a: Ascending, B: Descending) and branch vessels
+ - III: Descending thoracic and abdominal aorta ± renal arteries; may have atypical coarctation
+ - IV: Abdominal aortic ± renal arteries
+ - V: Entire aorta and its branches
+- ## Nuclear Medicine Findings
+
+
+ - ### PET
+
+
+ - FDG uptake; ranges from low grade to intense
+ - Treatment monitoring: Multiple qualitative and quantitative methods
+- ## Imaging Recommendations
+
+
+ - ### Protocol advice
+
+
+ - Multiplanar reconstructions for stenosis
+
+# DIFFERENTIAL DIAGNOSIS
+
+- [Giant Cell Arteritis](/document/giant-cell-arteritis/208eca17-81b8-448c-b8be-80e274dccc42)
+ - Affects large vessels in older patients (> 50 years)
+- [Vasculitis Mimics](/document/coarctation-of-aorta/c0b23d8c-05e3-4373-b5d9-2de1590414a7)
+ - Infections (tuberculosis, syphilis, HIV, bacterial)
+ - Atherosclerosis; thromboembolism
+ - Genetic disorders: Marfan, Ehlers-Danlos IV, Loeys-Dietz, Grange
+ - Congenital: Coarctation, Turner syndrome, Williams syndrome
+ - Unknown etiology: Fibromuscular dysplasia, segmental arterial mediolysis
+- [Behçet Disease](/document/behcet-syndrome/b08f488b-dd30-425e-9e5f-243c5feb477f)
+ - Large vessel vasculitis seen in 30% of these patients
+ - Proximal PA aneurysms are common
+- ## Other Causes of Aortitis
+
+
+ - Ankylosing spondylitis, rheumatoid arthritis, Cogan syndrome, relapsing polychondritis, IgG4-related disease
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Autoimmune etiology is suspected
+ - Infectious triggers suspected: Mycobacterium tuberculosis
+ - Suspected association with active or latent TB
+ - ### Genetics
+
+
+ - Specific types of human leukocyte antigen are common
+ - Strong association with HLA-B52
+- ## Gross Pathologic & Surgical Features
+
+
+ - Wall thickening of large vessels
+- ## Microscopic Features
+
+
+ - Granulomatous inflammation of arterial wall
+ - Intimal proliferation; fibrosis of media and adventitia
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - **Early or prepulseless phase**
+ - Low-grade fever, malaise, weight loss, fatigue
+ - **Vascular inflammatory phase**
+ - Vascular insufficiency
+ - Symptoms are minimized by collateral formation
+ - **Late quiescent occlusive or pulseless phase**
+ - Diminished/absent pulses, vascular bruits, claudication
+ - Blood pressure discrepancies in upper extremity
+ - Subclavian steal syndrome
+ - Hypertension, aortic regurgitation
+ - Neurologic symptoms: Headache, dizziness, seizures, stroke
+ - Ocular symptoms (ranging 8-68%): Hypertensive retinopathy or Takayasu retinopathy (due to hypoperfusion)
+ - Triphasic pattern is seen in minority of patients
+ - Disease is usually recurrent; phases may coexist
+ - Interval between early and late phases is variable
+ - **Cardiac involvement**
+ - Myocardial ischemia: 84% of asymptomatic patients
+ - Can cause angina, myocardial infraction, or sudden cardiac death
+ - Coronary involvement is independent predictor of poor long-term outcomes
+ - Type 1 (most common): Stenosis or occlusion of coronary ostia and proximal coronary artery
+ - Type 2: Diffuse or focal coronary arteritis (skip lesions)
+ - Type 3: Coronary aneurysms
+ - ### Other signs/symptoms
+
+
+ - Pulmonary hypertension when PA is involved
+ - Increases risk of early mortality
+- ## Demographics
+
+
+ - ### Age
+
+
+ - Most common in 2nd and 3rd decades of life
+ - ### Sex
+
+
+ - F:M = 8:1
+ - ### Epidemiology
+
+
+ - Most common in Asia
+ - Affects 6 out of 1,000 persons worldwide
+ - Annual incidence in USA: 2-3
+- ## Natural History & Prognosis
+
+
+ - Congestive heart failure is most common cause of death
+ - Hypertension is poor prognostic factor
+- ## Treatment
+
+
+ - Corticosteroids are 1st-line treatment; cyclophosphamide and methotrexate are 2nd line
+ - Angioplasty, surgical bypass, or stent placement for stenosis and occlusion
+
+ 37263d6e-e027-4379-8e09-1e15b44df3b6
+
+## References
+
+# Selected References
+
+1. [Somashekar A et al: Updates in the diagnosis and management of Takayasu's arteritis. Postgrad Med. 135(sup1):14-21, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36588528%5Bpmid%5D)
+1. [Grayson PC et al: 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis. Arthritis Rheumatol. 74(12):1872-80, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36349501%5Bpmid%5D)
+1. [Jia S et al: Application progress of multiple imaging modalities in Takayasu arteritis. Int J Cardiovasc Imaging. 37(12):3591-601, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34287748%5Bpmid%5D)
+1. [Chatterjee S et al: Clinical diagnosis and management of large vessel vasculitis: Takayasu arteritis. Curr Cardiol Rep. 16(7):499, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24893936%5Bpmid%5D)
+1. [Khandelwal N et al: Multidetector CT angiography in Takayasu arteritis. Eur J Radiol. 77(2):369-74, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=19720487%5Bpmid%5D)
+1. [Restrepo CS et al: Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta. Radiographics. 31(2):435-51, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21415189%5Bpmid%5D)
+1. [Pipitone N et al: Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford). 47(4):403-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18292120%5Bpmid%5D)
+1. [Desai MY et al: Delayed contrast-enhanced MRI of the aortic wall in Takayasu's arteritis: initial experience. AJR Am J Roentgenol. 184(5):1427-31, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15855090%5Bpmid%5D)
+1. [Tso E et al: Takayasu arteritis: utility and limitations of magnetic resonance imaging in diagnosis and treatment. Arthritis Rheum. 46(6):1634-42, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12115196%5Bpmid%5D)
+
+## Tables
+
+# Classification Criteria for Takayasu Arteritis: 2022 ACR/EULAR
+
+| | |
+| --- | --- |
+| Absolute Requirements |
+| Age ≤ 60 | |
+| Evidence of vasculitis on imaging | |
+| Additional Clinical Criteria |
+| Female sex | +1 |
+| Angina or ischemic cardiac pain | +2 |
+| Claudication | +2 |
+| Vascular bruit | +2 |
+| Diminished pulse in upper extremity | +2 |
+| Carotid artery abnormality | +2 |
+| Systolic BP difference in arms ≥ 20 mmHg | +1 |
+| Additional Imaging Criteria |
+| Number of affected artery territory | |
+| 1 arterial territory | +1 |
+| 2 arterial territories | +2 |
+| 3 or more arterial territories | +3 |
+| Symmetric involvement of paired arteries | +1 |
+| Abdominal aorta involvement with renal or mesenteric involvement | +3 |
+| Score of ≥ 5 points = Takayasu arteritis | |
+
+## Anatomy
+
+### Cervical Carotid Arteries
+Brain/ANATOMY:6eafa14e-2538-41b5-850c-29c41f38b970
+
+### Aortic Arch and Great Vessels
+Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
+
+### Pulmonary Vessels
+Chest/ANATOMY:551f4b47-fac1-44f9-b800-09ce766fedd9
+
+### Vessels, Lymphatic System, and Nerves, Abdominal
+Gastrointestinal/ANATOMY:0c38fd49-88e7-4272-960f-b17a194ce0cc
+
+### Carotid Space
+Head and Neck/ANATOMY:627bdee1-4bde-46f2-b93d-958882586337
+
+### Aorta and Inferior Vena Cava
+Ultrasound/ANATOMY:eed39e4d-478b-45d3-9406-1eace6e9eef1
+
+### Abdominal Aorta and Visceral Vasculature Anatomy
+Vasculature/ANATOMY:341c0af5-bfe8-4755-a401-39b7b9f2d9b9
+
+## Cases
+
+- {'cases': [{'authors': [{'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}, {'key': '3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1', 'value': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI'}], 'caseVersionId': '9dddd688-4ce9-4c92-b4c8-ec6f1ee538b0', 'description': 'Oblique axial volume rendered CECT (#1) shows severe narrowing (arrow) of the right interlobar pulmonary artery. Oblique sagittal VR CECT (#2) demonstrates moderate narrowing (arrow) of the proximal left lower lobe pulmonary artery. An oblique coronal VR CECT (#3) demonstrates severe narrowing of the right upper lobe (black arrow) and the interlobar pulmonary arteries (white arrow).', 'history': 'Patient with history of chronic dyspnea.', 'imagePoolId': '6e8f4a0f-9d6a-453e-8bb2-0f32a2b053eb', 'name': 'Takayasu arteritis', 'teachingPoint': None, 'demographics': '46 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial CECT in a patient with Takayasu arteritis (TA) shows marked thickening of the wall of the ascending thoracic aorta . The thoracic aorta and its branches, particularly the left subclavian artery, are the most commonly affected vessels in TA.*
+
+
+*Axial CECT in a patient with Takayasu arteritis (TA) shows marked thickening of the wall of the ascending thoracic aorta . The thoracic aorta and its branches, particularly the left subclavian artery, are the most commonly affected vessels in TA.*
+
+
+*Axial T1 MR in a patient with TA demonstrates thickening of the wall of the ascending thoracic aorta and the pulmonary trunk . Vessel stenosis, occlusion, and aneurysm formation may complicate cases of TA.*
+
+
+*Axial fused PET/CT in a patient with active TA demonstrates intense FDG uptake within the mediastinum adjacent to the aortic arch in a region of soft tissue attenuation that was present on the localization CT.*
+
+
+*Axial fused PET/CT in the same patient shows intense FDG uptake within the mediastinum adjacent to the ascending aorta and pulmonary arteries . FDG uptake may be low grade to intense in TA, and PET/CT is an effective way of monitoring treatment response.*
+
+
+*Composite image with axial CECT shows wall thickening of the proximal right brachiocephalic, left common carotid, and left subclavian arteries. There is more distal occlusion of the left common carotid and left subclavian arteries. The left subclavian artery is the most common branch vessel affected in patients with TA.*
+
+
+*Sagittal oblique DSA in another patient with TA shows patency of the left common carotid artery with occlusion of the right brachiocephalic and left subclavian arteries .*
+
+
+*Axial CECT in a patient with TA shows soft tissue attenuation and no contrast opacification within the left subclavian artery , consistent with occlusion.*
+
+
+*Sagittal oblique DSA in the same patient with TA shows occlusion of the left subclavian artery . Note the common origin of the right brachiocephalic and left common carotid arteries.*
+
+
+*Composite image with axial T1 MR (left) and DSA (right) shows aortic wall thickening and a focal aneurysm confirmed on subtraction aortic DSA .*
+
+
+*Axial CECT in a patient with TA shows an aortic dissection with marked intramural hemorrhage surrounding the false lumen . TA was confirmed on pathologic examination of the resected specimen.*
+
+
+### Additional Images
+
+
+*Axial T1 MR in a patient with TA shows abnormal high signal within the left common carotid artery , consistent with occlusion.*
+
+
+*Axial T1 MR in the same patient shows an absence of flow within the left subclavian artery , consistent with luminal occlusion.*
+
diff --git a/docs_md/articles/thoracic-aorta-and-great-vessel-anatomy_67498b94-770a-47ee-bd74-622b5e0b6817.md b/docs_md/articles/thoracic-aorta-and-great-vessel-anatomy_67498b94-770a-47ee-bd74-622b5e0b6817.md
new file mode 100644
index 0000000..9a6d64a
--- /dev/null
+++ b/docs_md/articles/thoracic-aorta-and-great-vessel-anatomy_67498b94-770a-47ee-bd74-622b5e0b6817.md
@@ -0,0 +1,278 @@
+---
+title: "Thoracic Aorta and Great Vessel Anatomy"
+docid: "67498b94-770a-47ee-bd74-622b5e0b6817"
+authors:
+ - key: "9fea2857-d729-4fe4-b4fd-3b7bf1db23cf"
+ value: "Mortadha Al-Kinani, MD, MBChB"
+ - key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
+ value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Anatomy"
+ slug: "anatomy"
+ treeNodeId: "d43d6d86-daf5-4eec-b2df-c88744ec7f32"
+ -
+ name: "Thoracic Aorta and Great Vessel Anatomy"
+ slug: "thoracic-aorta-and-great-vessel-an-"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "c2ccfda5-f93e-4770-bd79-17554ce23db9"
+imageCount: 21
+lastUpdated: "01/23/25"
+pageDescription: "Thoracic Aorta and Great Vessel Anatomy"
+pageKeywords: "Cardiac, Anatomy, Thoracic Aorta and Great Vessel Anatomy"
+pageTitle: "Thoracic Aorta and Great Vessel Anatomy | STATdx"
+enhancedTitle: "Thoracic Aorta and Great Vessel Anatomy"
+type: "ANATOMY"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Anatomy"
+ - "Thoracic Aorta and Great Vessel Anatomy"
+---
+# TERMINOLOGY
+
+- ## Definitions
+
+
+ - Aortic root
+ - Anulus to sinotubular junction (STJ)
+ - Ascending aorta (AsAo)
+ - Extends up to origin of brachiocephalic trunk
+ - Aortic arch or transverse aorta
+ - From brachiocephalic trunk to ligamentum arteriosum
+ - Ligamentum arteriosum is remnant of ductus arteriosus and typically lies immediately distal to origin of left subclavian artery (SCA)
+ - Aortic isthmus
+ - Segment of distal aortic arch between left subclavian origin and ligamentum arteriosum
+ - Descending thoracic aorta (DsAo)
+ - Ligamentum arteriosum to diaphragmatic hiatus
+
+# IMAGING ANATOMY
+
+- ## Overview
+
+
+ - Thoracic aorta divided into 4 segments from proximal to distal
+ - Aortic root
+ - AsAo
+ - Aortic arch
+ - DsAo
+ - Aortic root extends from aortic anulus to STJ
+ - Aortic anulus
+ - Virtual ring at base of aortic root defined by lowest attachment point of aortic cusps; cusp attachment site has complex crown shape
+ - Typically elliptical shape
+ - Important for sizing of aortic valve replacement
+ - Sinuses of Valsalva (SoVs)
+ - 3 sinuses defined by coronary origins
+ - Left coronary artery arises from left coronary sinus
+ - Right coronary artery arises from right coronary sinus
+ - Interatrial septum points toward noncoronary sinus, which is typically located posteriorly and to right on axial images
+ - SoV typically greatest caliber segment of thoracic aorta
+ - STJ
+ - Anatomic landmark dividing aortic root from tubular AsAo
+ - Narrower than SoVs
+ - AsAo extends from STJ to origin of brachiocephalic trunk
+ - Typically greatest in diameter and nearly orthogonal to axial plane at right pulmonary artery level, which is convenient and standard level of measurement
+ - Aortic arch extends from brachiocephalic trunk to ligamentum arteriosum
+ - Distal arch or aortic isthmus short (~ 2-cm) segment between left subclavian origin and remnant of ductus arteriosus
+ - Aortic isthmus typically narrower than adjoining aortic segments
+ - If ligamentum arteriosum cannot be identified, aortic arch can also be defined as extending past left subclavian origin
+ - Ductus diverticulum (or "bump"): Focal, smooth bulge at site of obliterated ductus arteriosus along undersurface of isthmus
+ - Normal variant that can be mistaken for traumatic aortic injury, which also occurs at this location
+ - May become aneurysmal (> 3 cm)
+ - Aortic arch branch vessels to head, neck, upper extremities, and chest wall termed great vessels
+ - Brachiocephalic trunk (innominate artery): 1st and largest of great vessels of aortic arch; divides into right common carotid artery (CCA) and SCA
+ - Right SCA branches include right internal mammary, vertebral artery (VA), thyrocervical, costocervical, and long thoracic arteries, and it continues as axillary artery after margin of 1st rib
+ - Right CCA divides into internal carotid artery (ICA) and external carotid artery (ECA) in neck
+ - Left CCA is 2nd great vessel from arch
+ - Divides into ICA and ECA
+ - Left SCA is 3rd and final great vessel from arch
+ - Gives off internal mammary, VA, thyrocervical, costocervical, and long thoracic arteries and continues as axillary artery
+ - Rare (3%) thyroid ima or thyroidea ima with inferior thyroid artery arises directly from aortic arch or innominate artery as opposed to normal origin from thyrocervical trunk
+ - DsAo extends from distal arch to diaphragmatic hiatus, where it continues as abdominal aorta
+ - Descending aorta typically smaller in caliber than AsAo
+ - Aortic spindle: Bulge in proximal descending aorta just distal to isthmus
+ - Commonly seen in children but can persist into adulthood
+ - Descending aorta gives off important small arteries
+ - Bronchial arteries
+ - Intercostal arteries
+ - Supreme intercostals supply T1-T3; arise from costocervical trunk of SCAs
+ - Paired intercostals arise directly from descending aorta from T4-T12
+ - Thoracic spinal cord supply comes from DsAo
+ - Anterior spinal artery supplied from intercostal and bronchial arteries at T4-T5
+ - Artery of Adamkiewicz arises from intercostal arteries at T6-T12 (75%)
+ - Esophageal, pericardial, superior phrenic, and other miscellaneous mediastinal branches
+ - Central venous anatomy
+ - Jugular veins
+ - Internal jugular veins drain head and neck; joined by external jugular veins draining face and scalp
+ - Subclavian veins
+ - Originate at axillary vein transition at 1st rib margin
+ - Typically valveless; joined by cephalic vein
+ - Brachiocephalic veins
+ - Formed by junction of subclavian and internal jugular veins
+ - Right: Short and vertical; left: Longer and crosses mediastinum anterior to great vessels
+ - Tributaries: Internal mammary, vertebral, pericardiophrenic, 1st intercostal, inferior thyroidal
+ - Superior vena cava (SVC)
+ - Formed by right and left brachiocephalic veins
+ - 6-8 cm long, up to 2 cm in diameter
+ - Azygos vein joins above pericardium; SVC enters right atrium
+- ## Anatomy Relationships
+
+
+ - Aortic arch variants
+ - Right aortic arch (< 0.1%); 2 types
+ - Mirror-image branching (65%); associated with cyanotic congenital heart disease in 90%
+ - Aberrant left SCA or other great vessel origin (35%); not associated with cyanotic congenital heart disease
+ - Dilated origin of aberrant left SCA in 60%; Kommerell diverticulum; if also ligamentum arteriosum → vascular ring and tracheal compression
+ - Double (duplicated) aortic arch (< 0.1%)
+ - Arises from 3rd rather than 4th branchial arch
+ - High location in chest, near lung apex
+ - May have anomalous great vessel origins
+ - Coarctation (< 0.1%)
+ - Congenital narrowing of aortic arch, usually distal to left subclavian origin
+ - May be preductal (infantile), juxtaductal, or post ductal (adult)
+ - Common with other congenital aortic pathology, such as bicuspid aortic valve and Turner syndrome
+ - Great vessel origin variants
+ - Bovine arch (20%): Left CCA may have common origin with or arise from innominate artery
+ - 4-vessel arch (5%): Left VA may arise directly from aortic arch between left CCA and left SCA rather than from left SCA
+ - Aberrant right SCA: Right SCA may arise separately from aortic arch, distal to left SCA
+ - Diverticulum of Kommerell: Dilatation at origin of aberrant right SCA; can be associated with dysphagia (dysphagia lusoria) when large
+
+# ANATOMY IMAGING ISSUES
+
+- ## Imaging Recommendations
+
+
+ - Thoracic aorta imaged with catheter angiography, transthoracic or transesophageal echocardiography, and CT or MR angiography
+ - CT angiography: Protocol may include noncontrast, arterial, and delayed-phase imaging
+ - Noncontrast images helpful in cases of extensive calcium, prior surgery, or suspicion for intramural hematoma
+ - Delayed images better delineate mediastinal anatomy and are also helpful in postsurgical patients when concern for endoleak
+ - Noncontrast and delayed images often not necessary for routine follow-up of known aortic aneurysm
+ - Thin-section (≤ 1.25-mm) reconstruction preferred
+ - ECG-gated or high-pitch dual-source CT preferred for accurate evaluation of aortic root due to cardiac motion artifact if root pathology suspected or followed
+ - MR angiography: Contrast angiography preferred
+ - Noncontrast sequences often give diagnostic study and are test of choice when contraindication to iodinated and gadolinium contrast agent
+ - In general, for follow-up exams, best to employ consistent imaging modality and measurement technique
+ - "Candy cane" oblique view places thoracic aorta in profile and is commonly employed for catheter, CT, and MR angiography
+ - Aortic measurement should be performed in plane orthogonal to longitudinal axis of aorta
+ - Measurements made in axial plane may be oblique to aorta and less accurate and reproducible
+- ## Transcatheter Aortic Valve Implantation/Replacement Assessment
+
+
+ - For severe aortic stenosis in nonsurgical patients
+ - Transfemoral or transapical approach may be chosen
+ - CT angiography plays increasing role in sizing of aortic anulus and determining suitability of iliofemoral approach
+ - Indications and criteria evolving
+ - PARTNER trial exclusion criteria
+ - Native aortic anulus size < 18 mm or > 25 mm
+ - Iliofemoral vessels too calcified or small to accommodate 22-Fr or 24-Fr introducer sheath (minimum luminal diameter of 7-8 mm, respectively)
+ - Severe aortic or iliofemoral disease that would preclude safe placement, such as aneurysm, tortuosity, extensive atheroma, or dissection
+ - Bulky, calcified aortic valve leaflets in close proximity to coronary ostia
+
+ c0614a38-3d8e-4373-8881-b9966f67112d
+
+## References
+
+# Selected References
+
+1. [Feldstein E et al: A novel variant of the aortic arch great vessels. Clin Neurol Neurosurg. 214:107172, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35158165%5Bpmid%5D)
+1. [Smith CR et al: Transcatheter versus surgical aortic-valve replacement in high-risk patients. N Engl J Med. 364(23):2187-98, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21639811%5Bpmid%5D)
+1. [Hiratzka LF et al: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. Circulation. 121(13):e266-369, 2010. Erratum in: Circulation. 122(4):e410, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20233780%5Bpmid%5D)
+1. [Agarwal PP et al: Multidetector CT of thoracic aortic aneurysms. Radiographics. 29(2):537-52, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19325064%5Bpmid%5D)
+1. [Leipsic J et al: The evolving role of MDCT in transcatheter aortic valve replacement: a radiologists' perspective. AJR Am J Roentgenol. 193(3):W214-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19696262%5Bpmid%5D)
+1. [Davies M et al: Developmental abnormalities of the great vessels of the thorax and their embryological basis. Br J Radiol. 76(907):491-502, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12857711%5Bpmid%5D)
+
+
+## Images
+
+
+### Thoracic Aorta and Great Vessels
+
+
+*Graphic depicts the thoracic aorta and great vessel origins.*
+
+
+*Graphic depicts the thoracic aorta and great vessel origins.*
+
+
+*Graphic depicts the branches of the descending thoracic aorta, including the intercostal, esophageal, and bronchial arteries. Typically, there are both superior and inferior left bronchial arteries and a single right bronchial artery (not pictured). Note that the left mainstem bronchus is bent anterior to allow depiction of the bronchial arteries.*
+
+
+### Normal Anatomy of Thoracic Aorta and Great Vessels
+
+
+*Frontal chest radiograph shows a normal thoracic aorta. The aortic knob shadow is created by a superimposition of the aortic arch and proximal descending aorta. The lateral margin of the descending thoracic aorta should always be visible, but the medial margin is usually not perceptible. The lateral margin of the ascending aorta is visible as part of the right mediastinal border.*
+
+
+*Corresponding frontal projection of a catheter angiogram (left) and digital subtraction image (right) of the thoracic aorta illustrate normal anatomy of the aorta and great vessel origins.*
+
+
+### Aortic Root CT Anatomy
+
+
+*"Candy cane" oblique MPR depicts the segments of the thoracic aorta. The aortic root (red) extends from the aortic anulus to the sinotubular junction. The ascending aorta (blue) extends to the origin of the brachiocephalic trunk. The aortic arch (yellow) extends to the ligamentum arteriosum. The descending thoracic aorta (green) extends to the diaphragmatic hiatus, where it continues as the abdominal aorta. Note the smooth outpouching along the inferior surface of the aortic arch at the remnant of the ductus arteriosum. This is a normal ductus diverticulum and should not be mistaken for a traumatic aortic injury.*
+
+
+*"Candy cane" view of an MRA shows a thoracic aorta.*
+
+
+*Three-chamber view from a CT angiogram depicts the anatomy of the left ventricular outflow tract and aortic root.*
+
+
+*Graphic depicts the most common configuration of the aortic arch, the 3-vessel arch.*
+
+
+*Graphic depicts common aortic arch variants. In the most common variant (upper left), the brachiocephalic trunk and left common carotid artery share a common origin. In the 2nd most common variant (upper right), the left common carotid artery arises from the brachiocephalic trunk. The left vertebral artery may arise directly from the aortic arch (lower left), between the left common carotid and subclavian arteries. The aberrant right subclavian artery arises from the distal aortic arch after the takeoff of the left subclavian artery (lower right), courses behind the trachea and esophagus to the right, and therefore may cause dysphagia (termed dysphagia lusoria).*
+
+
+*Digital subtraction catheter angiogram in the "candy cane" oblique view demonstrates 2 common aortic arch variants: Common origin of the brachiocephalic trunk and left common carotid (bovine arch) and a left vertebral artery arising directly from the aortic arch.*
+
+
+### Standard Measurements
+
+
+*Volume-rendered 3D CTA in "candy cane" view from a patient with a bicuspid aortic valve shows characteristic aneurysmal bowing of the ascending aorta. White lines denote standard aortic measurement planes, orthogonal to the long axis of the respective aortic segment. From proximal to distal, they include aortic anulus, sinus of Valsalva, sinotubular junction, ascending aorta at the level of the right pulmonary artery, aortic arch between the origins of the left subclavian and common carotid arteries, and the descending aorta.*
+
+
+*Multiplanar reformation of the aortic anulus shows the typical ovoid shape of the anulus. Accurate measurement of the anulus is important for sizing aortic valve replacements. Long- and short-axis diameters are reported. Annular circumference and area may also be helpful.*
+
+
+*Image immediately above the aortic anulus depicts portions of the aortic valve cusps. The anulus is measured as a virtual ring defined by the attachment of the lowest points of each aortic cusp.*
+
+
+### Aortic Root Short-Axis Planes
+
+
+*Multiplanar reformat through the sinus of Valsalva is shown. The sinus of Valsalva diameters are measured from commissure to cusp. Note that the interatrial septum points toward the noncoronary cusp in all projections. Inset shows the 3 diameter measurements (commissure to contralateral sinus) obtained in this plane.*
+
+
+*Oblique MIP through the sinus of Valsalva depicts the coronary origins.*
+
+
+*Oblique MPR, orthogonal to the aorta at the level of the sinotubular junction, shows that the sinotubular junction is of lower caliber than the sinus of Valsalva. Aortic diameters are most accurately and reproducibly measured in the plane orthogonal to the centerline of the aorta.*
+
+
+### Standard Planes of Aorta
+
+
+*Axial CT at the level of the right pulmonary artery shows the ascending and descending aorta. The ascending aorta is often greatest in diameter and nearly orthogonal to the axial plane at this level.*
+
+
+*MPR orthogonal to the aortic long axis at the level of the aortic arch, between the origins of the left common carotid and left subclavian arteries, is the standard plane for aortic arch diameter measurement.*
+
+
+*Oblique MIP in C view depicts the course of the right coronary artery and the origins of the left and right coronary arteries from the sinus of Valsalva.*
+
+
+### TAVI/R Planning
+
+
+*Curved multiplanar reformation high-pitch gated CTA shows the entire aorta and left iliofemoral system (right). Coned-down lateral radiograph shows a transcatheter aortic valve replacement (TAVR) (upper left inset). CTA is increasingly used for TAVR planning. The dimensions of the aortic anulus (mid left inset) are critical for valve sizing. The minimum luminal diameter of the iliofemoral arteries (lower left inset) and the degree of tortuosity and calcification of the aorta and iliofemoral system (right) determine whether a transfemoral approach is possible.*
+
+
+*Oblique MIP depicts the coronary artery origins. Obstruction of the coronary ostia by displaced aortic valve leaflets is an infrequent but reported complication of TAVR, so the distance from the aortic anulus plane (yellow line) to the closest coronary ostium is provided. In this case, the distance to the right coronary ostium corresponds to the double-headed black arrow.*
+
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@@ -0,0 +1,602 @@
+---
+title: "Thoracic Aortic Aneurysm"
+docid: "3c637054-d97c-4ae6-bc0c-ceac5f4a4f1f"
+authors:
+ - key: "b00d2bdb-66e1-41ed-90b4-c52904f4d598"
+ value: "Seth Kligerman, MD, MS"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Thoracic Aortic Aneurysm"
+ slug: "thoracic-aortic-aneurysm"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "2f92fee6-c59f-47c3-b87c-fcc3874adbf9"
+imageCount: 48
+lastUpdated: "02/10/25"
+pageDescription: "Thoracic Aortic Aneurysm"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Thoracic Aortic Aneurysm"
+pageTitle: "Thoracic Aortic Aneurysm | STATdx"
+enhancedTitle: "Thoracic Aortic Aneurysm"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Thoracic Aortic Aneurysm"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Aortic dilatation > 50% of normal diameter
+ - Etiology
+ - Heritable syndromes with multisystem features
+ - Heritable but nonsyndromic
+ - Congenital [more common in aortic aneurysm (AA) in younger patients]
+ - Degenerative [more common in descending thoracic aorta (DTA) in older patients]
+ - Inflammatory; infectious
+- ## Imaging
+
+
+ - Radiography
+ - Ascending AA: Curvilinear density along right aspect of mediastinum but often absent
+ - Aortic arch aneurysm: Enlarged/obscured aortic arch
+ - DTA aneurysm: Focal or diffuse abnormality of left paraaortic interface
+ - Peripheral curvilinear calcification
+ - Rupture: Wide mediastinum, left pleural effusion
+ - CT
+ - High spatial resolution allows for precise measurements
+ - AA often has no or limited atherosclerotic disease and more common in heritable and congenital cases
+ - DTA aneurysm commonly degenerative and associated with calcified and noncalcified atherosclerotic disease, often with mural thrombus
+ - MR
+ - Good for aortic measurements
+ - Excellent for assessment of aortic wall
+ - Evaluation of aortic valve and cardiac function
+ - PET/CT can help diagnose and monitor vasculitis
+- ## Pathology
+
+
+ - Variable depending on cause
+- ## Clinical Issues
+
+
+ - Repair of AA or DTA if ≥ 5.5 cm or smaller if rapid growth or symptomatic
+ - Repair criteria different based on syndromic and nonsyndromic heritable aneurysms or bicuspid aortic valve
+- ## Diagnostic Checklist
+
+
+ - Consider ruptured aneurysm: Acute chest pain, wide mediastinum, and pleural effusion on radiography
+ - Normal radiography does not exclude aneurysm or dissection; cross-sectional imaging for diagnosis
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Thoracic aortic aneurysm (TAA)
+- ## Definitions
+
+
+ - Aortic dilatation**> 50% of normal diameter**
+ - > 2 standard deviations (SDs) above mean
+ - Per 2022 American College of Cardiology (ACC)/American Heart Association (AHA) Guideline for Diagnosis and Management of Aortic Disease
+ - "Aneurysm" should be used when ascending aorta (AA) measures ≥ 4.5 cm due to significant increased risk of dissection
+ - For descending thoracic aorta (DTA), aneurysm would be classified at 1.5x mean diameter, which is ~ 4 cm
+ - "Dilated" should be used when AA diameter < 4.5 cm but > 2 SDs above mean for age, sex, and body surface area (BSA)
+ - Aneurysm definition is variable as "normal" size varies depending on age, sex, and associated conditions
+ - Diameter of aorta increases with age
+ - In one study of patients undergoing lung cancer screening, average and SDs of ascending aortic diameter was 3.21 ± 0.38, 3.35 ± 0.37, and 3.46 ± 0.35 for patients in age groups 55-59, 60-64, and 65-74 years, respectively
+ - Diameter is larger in men than women
+ - Men (wall-to-wall measurement)
+ - In 3 large studies, AA mean ranged from 34.1-36 mm
+ - In Framingham heart study, 41.9 mm was 2 SDs above mean
+ - For DTA [measured at level of main pulmonary artery (PA)] mean diameter was 25.8 ± 3.0 mm
+ - Women
+ - In 3 large studies, AA mean ranged from 31.9-33.5 mm
+ - In Framingham heart study, 38.9 mm was 2 SDs above mean
+ - For DTA (measured at level of main PA) mean diameter was 23.1 ± 2.6 mm
+ - Diameter will be larger if measuring entire wall-to-wall diameter vs. measuring only intraluminal area (IA)
+ - Men
+ - In one large study, wall-to-wall diameter > 2 SDs: 40.2, 42.9, and 45.0 mm in age groups 20-40, 41-60, > 60 years, respectively
+ - IA diameter > 2 SDs: 37.8, 40.5, and 42.6 mm in age groups 20-40, 41-60, > 60 years, respectively
+ - Women
+ - Wall-to-wall diameter > 2 SDs: 38, 40.7, and 42.4 mm for women in age groups 20-40, 41-60, > 60 years, respectively
+ - > 2 SDs for IA diameter: 35.6, 38.3, and 40 mm in age groups 20-40, 41-60, > 60 years, respectively
+ - Diameter is larger if measuring on axial image vs. MPR to get true transverse diameter
+ - In one study
+ - Men: Mean diameter decreases from 3.48 ± 0.36 to 3.41 ± 0.37 between axial and MPR measurements, respectively
+ - Women: Mean diameter decreases from 3.27 ± 0.36 to 3.22 ± 0.38 between axial and MPR measurements, respectively
+ - Diameter increases with increasing BSA
+ - e.g., in Framingham Heart Study for men > 65 years of age, mean AA diameter increased from 35.3 to 36.3 to 38.3 with BSA < 1.9, 1.9-2.09, and ≥ 2.1, respectively
+ - Similarly, for women 45-54 years of age, mean AA diameter increased from 29.7 to 31.5 to 32.5 with BSA < 1.9, 1.9-2.09, and ≥ 2.1, respectively
+ - Similar trends were seen across nearly all age groups and BSAs
+- ## Causes of Thoracic Aortic Aneurysms
+
+
+ - Heritable syndromes with multisystem features
+ - ~ 20% of TAAs due to genetic or heritable condition
+ - Marfan syndrome
+ - Aortic root dilation and type A dissection are major causes of morbidity and mortality in these patients
+ - Loeys-Dietz syndrome
+ - Vascular Ehlers-Danlos syndrome
+ - Smooth muscle dysfunction syndrome
+ - Others
+ - Heritable but nonsyndromic
+ - Mutations in *ACTA2*, *MYH11*, *PRKG1*, *MYLK*, and others
+ - Familial TAAs without identified pathogenic genetic variant known to cause heritable syndrome
+ - Congenital (more common in AA in younger patients)
+ - Bicuspid aortic valve (BAV)
+ - Turner syndrome
+ - Coarctation of aorta
+ - Complex congenital heart disease
+ - Degenerative (more common in DTA in older patients)
+ - Atherosclerosis
+ - Hypertension
+ - Inflammatory
+ - Vasculitis
+ - Giant cell arteritis
+ - Takayasu arteritis
+ - Behçet disease
+ - IgG4-related disease
+ - ANCA-related disease
+ - Sarcoidosis
+ - Infectious (pseudoaneurysm common)
+ - Bacterial
+ - Fungal
+ - Syphilitic
+ - Posttraumatic (pseudoaneurysm common)
+
+# IMAGING
+
+- ## Radiographic Findings
+
+
+ - Radiography
+ - **May not be visible (frequent)**
+ - Contour abnormality along aortic interface
+ - Aortic ascending aneurysm
+ - Abnormal convexity along superior right cardiomediastinal silhouette in frontal radiograph
+ - Fullness of retrosternal space in lateral radiograph
+ - Rightward tracheal &/or esophageal deviation
+ - Hilum overlay sign (in distal arch and descending aortic aneurysm)
+ - Lateralization of left paraaortic interface (in descending aortic aneurysm)
+ - Ruptured aneurysm
+ - Mediastinal widening compared with prior studies
+ - Pleural effusions
+- ## CT Findings
+
+
+ - Contrast-enhanced CT is excellent tool for aortic assessment
+ - ECG-gated preferred for AA assessment
+ - Aorta measured using multiplanar reconstructions as axial measurements overestimate size
+ - Typical landmarks include sinuses of Valsalva, sinotubular junction, maximum AA (often near level of main PA), distal AA, aortic arch, proximal DTA, mid-DTA, distal DTA near diaphragmatic hiatus
+ - Commonly used terms and definitions
+ - Aneurysm
+ - AA: ≥ 4.5 cm
+ - More commonly encountered in younger patients with little degenerative changes to AA, even with sporadic cases
+ - DTA: ≥ 4 cm
+ - Most common encountered in older patients with prominent atherosclerotic disease
+ - Dilation or ectasia
+ - AA: ≥ 4cm, < 4.5cm
+ - Morphology
+ - Fusiform
+ - Symmetric dilation of aorta
+ - Most common
+ - Saccular
+ - Asymmetric bulging of aorta
+ - Can be seen with both true aneurysm and pseudoaneurysm
+ - Often encountered in arch or proximal DTA
+ - Annuloaortic ectasia (AAE)
+ - Conspicuous dilation of aortic root and AA leading to effacement of sinotubular junction
+ - Associated with heritable syndromes
+ - Specific patterns and associations
+ - Marfan, vascular Ehlers-Danlos, Loeys-Dietz: AAE
+ - Marked tortuosity of vertebral arteries in Loeys-Dietz
+ - BAV: Variable morphology
+ - Root phenotype (20%)
+ - BAV with aortic regurgitation: Diffuse dilatation of aorta from root to arch
+ - Ascending phenotype (70%)
+ - BAV with aortic stenosis: Dilatation in tubular AA most common
+ - More common in Sievert type 1 BAV (fused raphe)
+ - Extended phenotype
+ - Dilation from aortic root through proximal half of aortic arch
+ - More common in Sievert type 0 BAV (true bicuspid without fused raphe)
+ - Degenerative: Most commonly affects DTA with calcified and noncalcified atherosclerotic disease
+ - Often associated with mural thrombus
+ - Can be crescentic or circumferential
+ - Contrast can invaginate between areas of mural thrombus and can mimic penetrating atherosclerotic ulcer
+ - However, contrast does not extend beyond confined of aortic wall with irregular mural thrombus
+ - Turner syndrome: Coarctation with aneurysm being less common
+ - Vasculitis: Aortic wall thickening with associated areas of aneurysmal dilation and stenosis; aortic wall enhancement may be present
+ - Infectious
+ - Bacterial and fungal: Saccular aneurysm with paraortic soft tissue stranding, fluid, or mass
+ - Often with rapid progression and pseudoaneurysm formation
+ - Signs of infection in adjacent structure
+ - Enhancement in aortic wall
+ - Syphilitic: Calcified ascending aortic aneurysm with circumferential wall thickening; wall can enhance on delayed imaging
+ - Rupture: Ill-defined aortic wall with surrounding mediastinal hematoma &/or hemothorax
+- ## MR Findings
+
+
+ - Similar findings as CT
+ - Obtained without use of radiation
+ - Similar specific patterns as described above
+ - Gated MRA should be performed if possible for root assessment
+ - Can measure aorta during different phases of cardiac cycle
+ - Aortic measurements will be larger during systole
+ - Additional value
+ - T2W imaging to assess for wall edema and plaque characterization
+ - T1W/T1W+ imaging to assess for wall enhancement and plaque characterization
+ - Delayed enhancement: Assess wall fibrosis (not commonly used)
+ - 4D flow: Assess aortic flow patterns
+- ## Nuclear Medicine Findings
+
+
+ - PET/CT
+ - Excellent tool for differentiating aortic inflammation or infection from other causes of TAA
+ - Helpful in monitoring for active disease
+- ## Imaging Recommendations
+
+
+ - Best imaging tool
+ - CT or MR for evaluation of aneurysm location and size, relationship to major branch vessels, and complications [e.g., dissection, mural thrombus, intramural hematoma (IMH), free rupture]
+ - Protocol advice
+ - ECG gating for anatomic and functional aortic valve assessment
+ - MR angiography can be performed with contrast and noncontrast techniques
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Tortuosity (Aging) of Aorta
+
+
+ - Diffuse aortic redundancy
+ - May require cross-sectional imaging for assessment
+- ## Mediastinal Mass
+
+
+ - Radiographic differentiation from neoplasm may be challenging and at times not possible
+ - Hilum overlay classic in anterior mediastinal masses
+ - Curvilinear calcification typical of vascular lesions
+- ## Pseudoaneurysm
+
+
+ - Contained rupture of aorta contained by piece of adventitia
+ - Extends beyond normal aortic wall
+ - Can mimic saccular aneurysm
+ - Common after previous aortic surgery, trauma, or infection
+ - Can have narrow or wide neck but extends beyond regular confines of aortic wall
+ - Often has surrounding inflammatory changes
+ - Disruption of intimal calcification with pseudoaneurysm
+- ## Acute Aortic Syndrome
+
+
+ - Aortic dissection (AD), IMH, and penetrating aortic ulcer (PAU) often occur in setting of TAA
+ - Acute aortic syndrome (AAS) is often symptomatic, whereas TAA is often asymptomatic unless ruptured or causing compression
+ - During acute stage, imaging findings of AAS can mimic certain findings seen with TAA
+ - Mural thrombus in TAA can mimic IMH in some instances
+ - In IMH, blood in wall of aorta will often have smooth crescentic shape but can be circumferential
+ - Contour is more irregular with mural thrombus
+ - Intramural blood in IMH is often more dense that layering mural thrombus in TAA
+ - Best seen on noncontrast imaging
+ - PAU can be difficult to differentiate from aneurysm with mural thrombus
+ - PAU extends beyond confines of aortic wall
+ - Discontinuity of atherosclerotic calcification often seen
+ - Surrounding hematoma may be present
+ - Additionally, imaging findings with healed AAS can also mimic findings seen with TAA
+ - False lumen of AD can thrombose and mimic aneurysm with mural thrombus
+ - Thrombosed false lumen usually has crescentic shape
+ - False lumen may be patent elsewhere along aorta
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - **True aneurysm**: Contains all 3 aortic wall layers
+ - **Atherosclerotic aortic aneurysm**
+ - Degenerative process, most common (75%)
+ - Old age, smoking, hypertension
+ - Shape: Fusiform > saccular
+ - Location: Most common in DTA
+ - **Infectious (mycotic) aneurysm**: Saccular; any location
+ - Predisposing causes: IV drug abuse, valvular disease, congenital aortic/cardiac disease, prior aortic/cardiac surgery, adjacent pyogenic infection, immunocompromise
+ - Most common pathogens: *Salmonella* spp. and *S.**aureus*
+ - Shape: Saccular
+ - Often pseudoaneurysms
+ - **Cystic medial necrosis**
+ - Degeneration of aortic media with medial necrosis
+ - Degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and interstitial collections of basophilic-staining ground substance
+ - Most commonly associated with syndromic and nonsyndromic heritable aneurysms
+ - However, also occurs in normal aging and accelerated by hypertension
+ - Shape: Fusiform
+ - Location: AA most common
+- ## Gross Pathologic & Surgical Features
+
+
+ - **Saccular**: Focal, mass-like aortic dilatation
+ - May result from remodeling of penetrating aortic ulcer
+ - **Fusiform**: Diffuse, elongated aortic dilatation
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - Most common signs/symptoms
+ - Atherosclerotic aortic aneurysm: Asymptomatic (most common), chest pain, compression (hoarseness, dysphagia, atelectasis, superior vena cava syndrome)
+ - Infectious (mycotic) aneurysm: Fever, leukocytosis
+ - Acute chest pain: Rupture, dissection
+- ## Demographics
+
+
+ - Age
+ - Atherosclerotic aortic aneurysms often free from significant atherosclerotic disease and more common in heritable and congenital cases
+ - Descending TAA more commonly degenerative and associated with calcified and noncalcified atherosclerotic disease
+ - Sex
+ - M > F
+ - Prevalence
+ - Increased from 3.5-7.6 per 100,000 persons between 2002-2014, primarily due to increased imaging
+- ## Treatment
+
+
+ - Risk reduction: Hypertension control, smoking cessation
+ - Indications for surgical or interventional aortic repair
+ - Sporadic or degenerative aneurysms
+ - **AA**≥ **5.5 cm**
+ - ≥ 5.0 cm reasonable if patient scheduled to undergo repair of trileaflet aortic valve
+ - **Descending aorta**≥ **5.5 cm**; smaller diameter threshold for repair include
+ - Symptomatic aneurysms
+ - Saccular aneurysm morphology
+ - Female
+ - Thoracic endovascular aortic repair (TEVAR) reasonable for patients who do not have syndromic aneurysm
+ - **Rapid growth**
+ - ≥ 0.5 cm in 1 year
+ - ≥ 0 .3 cm a year for 2 consecutive years
+ - Marfan syndrome
+ - Repair of any part of aorta if diameter > 5cm
+ - Repair of aortic root if diameter ≥ 4.5 cm is reasonable if there are high-risk features
+ - Family history of dissection
+ - Rapid growth
+ - Diffuse aortic root and AA dilation
+ - Marked vertebral artery tortuosity
+ - Cross-sectional aortic root area:patient height ratio ≥ 10 cm²/m
+ - Loeys-Dietz syndrome
+ - Prophylactic repair of aortic root and AA
+ - Size threshold, which ranges ≥ 4 cm to ≥ 5 cm based specific genetic variant, phenotypic features, patient age, aortic growth rates, and family history
+ - Vascular Ehlers-Danlos
+ - No specific guidelines due to increased surgical risk from vascular fragility and increased bleeding
+ - Decision should involve multidisciplinary team
+ - Heritable but nonsyndromic
+ - Diameter ≥ 5.0 cm in absence of high-risk features
+ - ≥ 4.5 cm in presence of high-risk features
+ - Family history of dissection at aortic diameter < 5.0 cm
+ - Unexplained sudden death at age < 50 years
+ - Rapid aortic growth defined as ≥ 0.5 cm in 1 year or ≥ 0.3 cm per year in 2 consecutive years
+ - BAV
+ - Aortic diameter ≥ 5.5 cm
+ - Repair of aortic root if diameter 5-5.4 cm is reasonable if there are high-risk features
+ - Family history of dissection
+ - Growth rate > 0.3 cm per year
+ - Aortic coarctation
+ - Root phenotype with isolated root dilation
+ - Aortic diameter ≥ 4.5 cm at time of aortic valve replacement or repair
+ - Recommend screening of all 1st-degree relatives via echocardiogram
+ - Surgical repair
+ - Open repair: Artificial or biologic grafts
+ - Closed repair: Endovascular stent graft
+- ## Imaging Follow-Up
+
+
+ - Nonsyndromic: Annual CTA or MRA
+ - Marfan syndrome: Yearly CT/MR if stable; more frequently if > 4.5 cm/growth
+ - Loeys-Dietz: Yearly MRA
+ - Turner syndrome: Every 5-10 years if no risk factors; yearly if abnormalities
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - Ruptured aneurysm in patients with acute chest pain, wide mediastinum, and pleural effusion on radiography
+ - Normal radiography does not exclude aneurysm or dissection
+ - Cross-sectional imaging is used for diagnosis
+- ## Reporting Tips
+
+
+ - AAE (blunt sinotubular junction) suggests Marfan syndrome; affects surgical procedure
+ - Assess coronary arteries and great vessels in cases with associated dissection
+
+ baa5b128-9459-450c-a064-f49badc6d92f
+
+## References
+
+# Selected References
+
+1. [Rodríguez-Palomares JF et al: Mechanisms of aortic dilation in patients with bicuspid aortic valve: JACC state-of-the-art review. J Am Coll Cardiol. 82(5):448-64, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37495282%5Bpmid%5D)
+1. [Isselbacher EM et al: 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation. 146(24):e334-482, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36322642%5Bpmid%5D)
+1. [Wang J et al: Aortic dilatation in patients with bicuspid aortic valve. Front Physiol. 12:615175, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34295254%5Bpmid%5D)
+1. [Kallianos KG et al: Imaging thoracic aortic aneurysm. Radiol Clin North Am. 58(4):721-31, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32471540%5Bpmid%5D)
+1. [Rooprai J et al: Thoracic aortic aneurysm growth in bicuspid aortic valve patients: role of aortic stiffness and pulsatile hemodynamics. J Am Heart Assoc. 8(8):e010885, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30966855%5Bpmid%5D)
+1. [McComb BL et al: Normative reference values of thoracic aortic diameter in American College of Radiology Imaging Network (ACRIN 6654) arm of National Lung Screening Trial. Clin Imaging. 40(5):936-43, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27203287%5Bpmid%5D)
+1. [Erbel R et al: 2014 ESC guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J. 35(41):2873-926, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25173340%5Bpmid%5D)
+1. [Goldfinger JZ et al: Thoracic aortic aneurysm and dissection. J Am Coll Cardiol. 64(16):1725-39, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25323262%5Bpmid%5D)
+1. [Rogers IS et al: Distribution, determinants, and normal reference values of thoracic and abdominal aortic diameters by computed tomography (from the Framingham Heart Study). Am J Cardiol. 111(10):1510-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23497775%5Bpmid%5D)
+1. [Mao SS et al: Normal thoracic aorta diameter on cardiac computed tomography in healthy asymptomatic adults: impact of age and gender. Acad Radiol. 15(7):827-34, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18572117%5Bpmid%5D)
+1. [Atar E et al: MR angiography for abdominal and thoracic aortic aneurysms: assessment before endovascular repair in patients with impaired renal function. AJR Am J Roentgenol. 186(2):386-93, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16423943%5Bpmid%5D)
+1. [Isselbacher EM et al: Thoracic and abdominal aortic aneurysms. Circulation. 111(6):816-28, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15710776%5Bpmid%5D)
+1. [Hager A et al: Diameters of the thoracic aorta throughout life as measured with helical computed tomography. J Thorac Cardiovasc Surg. 123(6):1060-6, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12063451%5Bpmid%5D)
+1. [Guo D et al: Familial thoracic aortic aneurysms and dissections: genetic heterogeneity with a major locus mapping to 5q13-14. Circulation. 103(20):2461-8, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11369686%5Bpmid%5D)
+1. [LePage MA et al: Aortic dissection: CT features that distinguish true lumen from false lumen. AJR 177:207-11, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11418429%5Bpmid%5D)
+1. [Posniak HV et al: CT of thoracic aortic aneurysms. Radiographics 10:839-55, 1990](http://www.ncbi.nlm.nih.gov/pubmed/?term=2217974%5Bpmid%5D)
+1. [Feigl D et al: Mycotic aneurysms of the aortic root. A pathologic study of 20 cases. Chest. 90(4):553-7, 1986](http://www.ncbi.nlm.nih.gov/pubmed/?term=3757565%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*PA radiograph in a 73-year-old man with a history of bicuspid valve status post repair shows abnormal right-sided convexity along the superior cardiomediastinal silhouette , suggestive of an ascending aortic (AA) aneurysm. In this case, atherosclerotic calcifications allow for partial visualization of the AA.*
+
+
+*PA radiograph in a 73-year-old man with a history of bicuspid valve status post repair shows abnormal right-sided convexity along the superior cardiomediastinal silhouette , suggestive of an ascending aortic (AA) aneurysm. In this case, atherosclerotic calcifications allow for partial visualization of the AA.*
+
+
+*Coronal CECT MIP in the same patient shows the AA atherosclerosis . The AA is aneurysmal with a maximum diameter of 5 cm.*
+
+
+*Lateral radiograph shows a prosthetic aortic valve with surrounding calcification of the aortic root and AA . A portion of the more superior AA can be partially visualized due to atherosclerosis and adjacent surgical clips from prior aortotomy .*
+
+
+*Sagittal CECT MIP shows the contours of the AA in related to the sternum and other mediastinal structures. While the AA is aneurysmal with a maximum transverse diameter of 5 cm, the aortic arch and descending thoracic aorta (DTA) are normal in size.*
+
+
+*Coronal oblique arterial-phase CECT in a 38-year-old with annuloaortic ectasia shows balloon-like AA dilation and effacement of the sinotubular junction. There is rapid tapering of the AA in its midportion. Subsequent work-up diagnosed Marfan syndrome.*
+
+
+*Coned down 3-chamber echocardiogram in a 36-year-old with Marfan syndrome shows balloon-like dilation of the AA above the aortic valve . During diastole, there is severe aortic regurgitation with flow paralleling adjacent mitral inflow .*
+
+
+*Axial CECT images from the years 2004, 2009, 2015, and 2022 are shown. The AA grew from 4.1 cm to 4.3 cm to 4.5 cm to 5.4 cm, respectively. Similarly, the DTA grew from 2.7 cm to 3 cm to 3.4 cm to 4.2 cm.*
+
+
+*Parasagittal (left) and coronal oblique (right) images through the aorta in a 79-year-old show diffuse fusiform atherosclerotic aneurysm of the DTA with extensive layering mural thrombus with areas of calcification due to degenerative changes. The maximum aortic diameter was 6.8 x 6.3 cm. The AA is normal.*
+
+
+*Oblique sagittal chest CECT in the same patient shows 2 saccular aneurysms with rather extensive atherosclerotic changes of the thoracic aorta.*
+
+
+*4D flow MR in a patient with a Sievert type 1 bicuspid aortic valve and aortic regurgitation shows vortical flow in the aneurysmal AA . The entire AA is enlarged.*
+
+
+*Coronal 5-mm MIP (left) shows circumferential thickening of the AA, which is irregularly dilated to 4.8 cm, not including the wall thickening . Sagittal image (right) shows the AA wall thickening as well as areas of wall thickening and mild stenosis in the abdominal aorta .*
+
+
+*Axial images from a CTA (left) and PET/CT (right) show intense FDG uptake in the wall of the AA . The patient was diagnosed with large vessel vasculitis.*
+
+
+*AP chest x-ray in a 58-year-old man admitted for deteriorating mental status over 2 months shows prominence of aortic contour . It was unclear if this was related to tortuosity or aneurysm.*
+
+
+*Subsequent CTA shows circumferential wall thickening and aneurysmal dilation of the AA measuring 5 cm. Wall thickening extends into the DTA but is more mild without dilation. Thickening extends around the ostia of the arch vessels with severe stenosis or the left common carotid artery .*
+
+
+*Sagittal T1W (L), T1W+ (R), and axial T1W+ (inset) spine MR images show diffuse aortic wall enhancement . CFS-VDRL was 1:1024, diagnosing neurosyphilis. Aorta has a typical appearance for syphilitic aortitis.*
+
+
+*Coronal (L) and sagittal oblique (center) images show a penetrating atherosclerotic ulcer (PAU) with hematoma extending beyond the aorta wall . Adjacent intimal calcification is absent in PAU region. Six years later (R), PAU has increased in size, representing a pseudoaneurysm.*
+
+
+*Axial CT in a 74-year-old man with chest pain shows a large volume of mediastinal hematoma compressing the left atrium. Hemothorax is also present. The DTA is aneurysmal with calcified and noncalcified atherosclerotic disease.*
+
+
+*Image more inferiorly in the same patient shows mediastinal hematoma and large right hemothorax . The DTA is aneurysmal with atherosclerotic disease and mural thrombus . Focal rupture of the DTA in this region was confirmed on catheterization.*
+
+
+*Aortic root images in a patient with Marfan syndrome and AA graft repair show aneurysmal dilation of the coronary artery button grafts (BGs) , which have increased in size between the years 2004 and 2020. The BGs are from the native aorta and thus susceptible to aneurysm formation.*
+
+
+*Axial (left) and sagittal oblique (right) CECT images of the thoracic aorta in a 73-year-old show extensive fusiform aneurysmal dilation of the DTA measuring up to 5.3 cm with diffuse layering mural thrombus .*
+
+
+*PA radiograph in a patient with severe emphysema and small left effusion shows a large mass in the superior mediastinum displacing the trachea and esophagus rightward. The location and rounded appearance could be due to an aneurysm.*
+
+
+*Coronal CTA in the same patient shows a large fusiform aneurysm of the proximal DTA displacing the esophagus rightward.*
+
+
+### Additional Images
+
+
+*Sagittal CTA shows a fusiform aneurysm of the descending aorta with a large amount of eccentric anterior endoluminal thrombus and posterior partially calcified atherosclerotic plaque .*
+
+
+*PA chest radiograph shows a focal left superior mediastinal mass that obscures the superior aspect of the left paraaortic interface . AAs may mimic nonvascular mediastinal masses.*
+
+
+*Frontal aortogram in the same patient shows that the mass represents a polylobular saccular aneurysm of the descending aorta. Posttraumatic, infectious, and atherosclerotic aneurysms would be considered in the differential diagnosis.*
+
+
+*PA radiograph of the chest in a patient with distal DTA aneurysm shows lateral displacement of the left paraaortic interface .*
+
+
+*Lateral chest radiograph in the same patient optimally demonstrates the extensive calcification and dilatation of the ascending aorta and a normal descending aorta. The differential diagnosis should also include syphilis (luetic aortitis) and type II hyperlipidemia.*
+
+
+*PA chest radiograph in a patient with prior trauma shows dilatation of the ascending aorta with a thick rim of curvilinear calcification .*
+
+
+*Lateral radiograph of the chest in the same patient shows dilatation of the distal thoracic aorta . While intervention of an ascending AA is often recommended at 5.5 cm (5.0 cm for Marfan syndrome and bicuspid aortic valve), a DTA aneurysm is generally repaired if > 6.5 cm.*
+
+
+*PA radiograph of the chest in a patient with proximal DTA aneurysm shows an abnormal contour overlying the left hilum , the so-called hilum overlay sign.*
+
+
+*Lateral chest radiograph in the same patient shows marked tortuosity and dilatation of the proximal descending aorta . Given the high risk of rupture, all aneurysm exceeding 6.5 cm along the descending thoracic aorta require intervention.*
+
+
+*Lateral chest radiograph in the same patient shows fullness of the retrosternal clear space. While this findings is nonspecific, it is frequently seen in anterior mediastinal masses, including ascending AAs.*
+
+
+*Oblique sagittal SSFP MR of the chest in a patient with bicuspid aortic valve and ascending AA shows ascending aortic dilatation with preservation of the sinotubular junction , a feature that helps differentiate from dilatation due to Marfan syndrome.*
+
+
+*Oblique contrast-enhanced 3D MRA of the chest was performed in the same patient. Of all sequences, MRA provides the best overview of aneurysm extent, although motion artefact may cause some blurring of the aortic root.*
+
+
+*PA chest radiograph in a patient with atherosclerotic aneurysm of the ascending aorta shows mediastinal widening. Note that the ascending aorta overlies the right hilum, a sign concerning for mediastinal mass. Often, chest radiography is not sensitive enough to detect this abnormality.*
+
+
+*Axial CTA of the chest in the same patient demonstrates marked dilatation of the ascending aorta . Note an atherosclerotic plaque along the descending aorta.*
+
+
+*Axial chest CECT in the same patient shows dilated distal ascending thoracic aorta . Note that there is thin intraluminal thrombus within the aneurysm. Also note that the pulmonary trunk exhibits a discordant diameter .*
+
+
+*PA chest radiograph in a patient with a saccular aneurysm involving the distal aortic arch shows abnormal convexity with linear calcification occupying the AP window. An abnormal mediastinal contour abnormality with peripheral curvilinear calcification is a common radiographic appearance in AA.*
+
+
+*Axial chest CECT in the same patient shows saccular aneurysm involving the aortic arch. Note that the curvilinear calcification surrounding the aneurysm.*
+
+
+*Coronal chest CECT in the same patient shows fusiform dilatation of the ascending aorta with preservation of the sinotubular junction . Effacement of the sinotubular junction is frequently seen in the setting of Marfan syndrome.*
+
+
+*PA chest radiograph in a patient with saccular aneurysm involving the distal ascending aorta and proximal aspect of the aortic arch shows abnormal convexity along the superior cardiomediastinal silhouette . Abnormal cardiomediastinal contour abnormalities are always concerning of mediastinal mass, among which aneurysm is a common etiology.*
+
+
+*Oblique coronal chest CECT in the same patient shows a saccular aneurysm involving the distal ascending thoracic aorta and proximal aortic arch. Saccular aneurysms are thought to be related to remodeling of penetrating aortic ulcers undiagnosed during the acute setting.*
+
+
+*Axial NECT in the same patient reveals a mediastinal hematoma with the crescent sign along the DTA and left pleural effusion . These 3 CT signs are all associated with aortic rupture, which (when present) constitutes an indication of emergent repair.*
+
+
+*Axial CTA in the same patient shows a DTA aneurysm with an intraluminal thrombus , periaortic hematoma , mediastinal hematoma , and hemothorax .*
+
+
+*AP radiograph of the chest in a patient with a ruptured aneurysm of the aorta shows marked mediastinal widening and a moderate-sized left pleural effusion .*
+
+
+*Axial NECT of the chest in the same patient also shows an aneurysm of the proximal DTA associated with high-attenuation pleural fluid as well as a hematocrit-fluid level, indicating rupture and hemothorax .*
+
+
+*Axial CTA of the chest in the same patient additionally shows an aneurysm of the proximal DTA associated with extensive intraluminal thrombus .*
+
+
+*Graphic shows the Crawford classification of aortic aneurysms. Type I affects the descending thoracic and proximal abdominal aorta, type II the entire descending thoracic and abdominal aorta, type III the distal descending and abdominal aorta, and type IV the abdominal aorta.*
+
diff --git a/docs_md/articles/traumatic-aortic-injury_061b04b5-e37e-4f63-a198-1020a984e041.md b/docs_md/articles/traumatic-aortic-injury_061b04b5-e37e-4f63-a198-1020a984e041.md
new file mode 100644
index 0000000..89f436b
--- /dev/null
+++ b/docs_md/articles/traumatic-aortic-injury_061b04b5-e37e-4f63-a198-1020a984e041.md
@@ -0,0 +1,481 @@
+---
+title: "Traumatic Aortic Injury"
+docid: "061b04b5-e37e-4f63-a198-1020a984e041"
+authors:
+ - key: "770e1d77-2287-436e-910b-48232afc7842"
+ value: "Prabhakar Rajiah, MBBS, MD, FACR, FRCR, FACC, FAHA, FSCCT"
+ - key: "8b719ccc-d695-4377-9afb-2b399714d382"
+ value: "Terrance Healey, MD"
+breadcrumbs:
+ -
+ name: "Cardiac"
+ slug: "cardiac"
+ treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
+ -
+ name: "Aorta"
+ slug: "aorta"
+ treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
+ -
+ name: "Traumatic Aortic Injury"
+ slug: "traumatic-aortic-injury"
+ treeNodeId: null
+category: "Cardiac"
+documentVersionId: "cfab5ac9-7423-43eb-9721-be17555dfc4c"
+imageCount: 21
+lastUpdated: "11/26/24"
+pageDescription: "Traumatic Aortic Injury"
+pageKeywords: "Cardiac, Diagnosis, Aorta, Traumatic Aortic Injury"
+pageTitle: "Traumatic Aortic Injury | STATdx"
+enhancedTitle: "Traumatic Aortic Injury"
+type: "DX"
+references: true
+anatomy:
+ - "{'authors': 'Anne G. Osborn, MD, FACR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'category': 'Brain', 'compareUrl': '/compare/document/a7a252f0-2ac6-402a-8c87-cfce8adc799b/related-anatomy/treeNode?subContext=Aortic Arch and Great Vessels', 'documentId': 'a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'documentType': 'ANATOMY', 'documentUrl': '/document/aortic-arch-and-great-vessels/a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'enhancedTitle': 'Aortic Arch and Great Vessels', 'entryDate': '10/20/20', 'imageCount': 8, 'imageUrl': '/image/thumbnail/5a451d53-7fe4-426e-b816-7f0e0a1df745?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Aortic Arch and Great Vessels'}"
+cases: 2
+breadcrumbs:
+ - "Cardiac"
+ - "Diagnosis"
+ - "Aorta"
+ - "Traumatic Aortic Injury"
+---
+# KEY FACTS
+
+- ## Terminology
+
+
+ - Traumatic injury of aorta (TAI) as result of motor vehicle collision (MVC), fall, or, less commonly, penetrating trauma
+ - Synonyms: Acute TAI, blunt traumatic aortic rupture, blunt aortic trauma, blunt aortic injury, aortic transection, traumatic aortic pseudoaneurysm
+ - Minimal aortic injury (MAI)
+- ## Imaging
+
+
+ - Radiography
+ - Wide mediastinum: Hematoma, exclusion of TAI
+ - 1st rib fracture: Severe trauma, possible TAI
+ - CTA: Imaging modality of choice
+ - Aortic isthmus (90%); commonly on inferomedial aspect
+ - Direct: Intramural hematoma; intimal flap; pseudoaneurysm
+ - Indirect: Periaortic hematoma; irregular aortic contour; sudden aortic caliber change
+ - Sensitivity 98%; specificity 80%
+- ## Top Differential Diagnoses
+
+
+ - Wide mediastinum of other etiology
+ - Mediastinal hematoma: Other causes
+ - Ductus diverticulum (type III)
+ - Infundibulum of bronchial-intercostal trunk
+ - Atherosclerotic ulceration
+ - Fusiform enlargement proximal descending aorta
+- ## Clinical Issues
+
+
+ - No specific or sensitive signs or symptoms until hemodynamic instability ensues
+ - Urgent diagnosis; 50% die within 24 hours if untreated
+ - Cause of death in 20% of high-speed MVCs
+ - Treatment
+ - Surgical repair: 70-85% survival (up to 20% surgical mortality)
+
+# TERMINOLOGY
+
+- ## Abbreviations
+
+
+ - Traumatic aortic injury (TAI)
+- ## Synonyms
+
+
+ - Acute TAI (ATAI)
+ - Aortic transection
+ - Blunt aortic injury (BAI)
+ - Blunt aortic trauma (BAT)
+ - Blunt traumatic aortic rupture (BTAR)
+ - Traumatic aortic pseudoaneurysm
+ - Minimal aortic injury (MAI)
+ - Significant aortic injury (SAI)
+- ## Definitions
+
+
+ - Traumatic injury of aorta as result of motor vehicle collision (MVC), fall or, less commonly, penetrating trauma
+ - Partial tear vs. complete rupture
+
+# IMAGING
+
+- ## General Features
+
+
+ - ### Best diagnostic clue
+
+
+ - **Widened mediastinum** on AP chest radiography
+ - **Intramural hematoma (IMH), intimal flap,** **pseudoaneurysm or rupture** on CTA
+ - ### Location
+
+
+ - **Aortic isthmus (90% of initial survivors)**; commonly along inferomedial aspect at level of left pulmonary artery
+ - Ascending aorta (5-14% of initial survivors)
+ - Most die at scene of accident
+ - Diaphragmatic hiatus (1-12%)
+ - May be associated with diaphragmatic injury
+ - Multiple sites rarely affected
+ - ### Morphology
+
+
+ - SAI
+- ## Radiographic Findings
+
+
+ - ### Radiography
+
+
+ - **Indirect signs** related to mediastinal hemorrhage
+ - Signs of TAI: Sensitive but not specific
+ - Most signs present in 30-70% of patients
+ - **Widened superior mediastinum** (> 8 cm or > 25% of transthoracic diameter)
+ - **Left apical pleural****cap**
+ - Abnormal aortic arch contour
+ - Obscuration of AP window
+ - Right tracheal &/or endotracheal tube shift
+ - Right enteric tube shift
+ - Wide paravertebral stripe
+ - Wide right paratracheal stripe
+ - Inferior displacement of left mainstem bronchus
+ - 1st rib fracture indicates severe trauma and possible TAI
+ - 1st rib protected by clavicle and scapula, requires considerable force to break
+ - Frequency of TAI is 15-30%
+ - Any of aforementioned signs requires further investigation to exclude aortic transection
+ - Normal chest radiograph (7%)
+ - Chronic pseudoaneurysm (2% of survivors)
+ - Peripherally calcified mass at aorticopulmonary window
+- ## CT Findings
+
+
+ - ### NECT
+
+
+ - Often shows mediastinal hematoma, rarely shows site of tear
+ - ### CTA
+
+
+ - Imaging modality of choice
+ - Direct visualization of aortic tear, markedly reducing need for aortography
+ - Sensitivity: 98%, specificity: 80%
+ - **Direct signs**
+ - IMH
+ - **Intimomedial flap ± thrombus**
+ - **Pseudoaneurysm or contained rupture**
+ - Rarely complete rupture with active extravasation
+ - Aortic dissection
+ - Indirect signs
+ - Periaortic/mediastinal hematoma
+ - Irregular aortic contour
+ - Sudden aortic caliber change; pseudocoarctation: Best seen on MPR
+ - MAI: Absent contour abnormality
+ - 10% of acute TAI (ATAI)
+ - Increased diagnosis due to improved spatial resolution of CT
+ - Intimal flap (small < 1 cm; large > 1 cm) ± thrombus; IMH without contour abnormality
+ - May remain stable or resolve
+ - SAI: Contour abnormality present
+ - IMH + contour abnormality; pseudoaneurysm (< or > 1 cm); aortic rupture
+ - More severe injuries: Have both direct and indirect findings
+ - Pitfalls of TAI
+ - Pulsation or streak artifact, especially at aortic root
+ - ECG gating required
+ - Ambiguous findings
+ - Use different slice thickness, imaging plane, reconstruction kernel, or different phase of cardiac cycle
+ - Mimics of TAI
+ - Ductus diverticulum; infundibulum; other causes of mediastinal hematoma; atherosclerotic plaque
+ - Chronic traumatic pseudoaneurysm
+ - Develop in undiagnosed or untreated injuries
+ - At isthmus; with extensive peripheral calcification; ± thrombus
+ - Calcification protects against rupture
+- ## MR Findings
+
+
+ - MR generally has no role in evaluation of acute trauma
+ - Limited by issues related to transportation and monitoring of critically injured patients
+ - Used to identify IMH in stable patients and for follow-up
+- ## Echocardiographic Findings
+
+
+ - Transesophageal ECG
+ - Demonstration of intimal tear, transection
+ - Visualization of hemopericardium
+ - May be technically difficult to perform in severely injured patients
+ - Most commonly used intraoperatively when CT cannot be performed
+- ## Angiographic Findings
+
+
+ - Angiography
+ - Previously considered gold standard for evaluating aorta and great vessels
+ - Sensitivity: 100%
+ - Specificity: 98%
+ - Using chest radiography as guide, 10 negative angiograms performed for each TAI diagnosed
+ - Small risk of rupture
+- ## Imaging Recommendations
+
+
+ - ### Best imaging tool
+
+
+ - **CTA is imaging modality of choice**
+ - ### Protocol advice
+
+
+ - Thin slices, MPR, and 3D volume rendering essential for treatment planning
+ - ECG gating required for evaluation of aortic root and ascending aorta
+
+# DIFFERENTIAL DIAGNOSIS
+
+- ## Widened Mediastinum on Chest Radiograph
+
+
+ - False-positives: Rotation, supine positioning, expiratory imaging, mediastinal fat
+- ## Mediastinal Hematoma: Other Causes
+
+
+ - Caused by injury to mediastinal veins, great vessels, pulmonary arteries, or vertebral body fractures
+ - Arterial injury not identified
+ - Hematoma usually away from aorta with intact fat plane
+- [Ductus Diverticulum (Type III)](/document/ductus-diverticulum/9b1101bc-83a2-445c-aef5-53d633e5bec0)
+ - Remnant of closed or partially closed ductus arteriosus
+ - Inferomedial outpouching of aortic isthmus
+ - Smooth, gently sloping shoulders with obtuse angle with aortic wall
+ - Often calcified; no intimal flap
+- ## Infundibulum of Bronchial-Intercostal Trunk
+
+
+ - Takeoff may show bump in aortic contour
+ - Cone-shaped, smooth walled, with artery at its apex
+- ## Atherosclerotic Ulceration
+
+
+ - More common in older patients
+ - Other coexisting aortic plaques
+- ## Normal-Variant Fusiform Dilation of Proximal Descending Aorta
+
+
+ - No intimomedial flap
+
+# PATHOLOGY
+
+- ## General Features
+
+
+ - ### Etiology
+
+
+ - Theories of pathogenesis
+ - Rapid deceleration injury with shearing forces greatest at levels of aortic immobility: Ligamentum arteriosum, aortic root, and diaphragmatic hiatus
+ - Lateral direction in side-impact collisions; anteroposterior direction in head-on collisions
+ - Osseous pinch: Aorta compressed between anterior chest wall (manubrium, medial clavicles, 1st rib) and spine; transverse tear at aortic isthmus
+ - Water hammer effect: Sudden marked increase in intravascular pressure during aortic compression; transverse tear at isthmus
+ - Multivariate hypothesis likely: Shearing, torsion, stretching, hydrostatic forces
+- ## Gross Pathologic & Surgical Features
+
+
+ - 90% at aortic isthmus
+ - From origin of left subclavian artery to ligamentum arteriosum, often anteromedially
+ - 7-8% involve aortic root; 2% involve descending aorta at diaphragmatic hiatus
+ - Ascending aortic tear: 20% of coroner cases; rarely survive long enough to reach hospital
+ - In autopsy series, only 45-58% involve isthmus compared to 95% in surgical series
+ - In abdominal aorta: Infrarenal segment is commonly affected
+ - Lap belt compression
+ - Associations: Lumbar spine fracture, pelvic fracture, injury to bowel, solid organs, spleen
+ - Range: Intimal hemorrhage to complete transection
+ - Transverse tears: Segmental (55%) or circumferential (45%); partial (65%) or transmural (35%)
+ - Noncircumferential tears more common posteriorly
+ - May involve aortic layers to varying degrees
+ - Survivors: Pseudoaneurysm usually contained by adventitia, or, occasionally, mediastinal structures
+ - Adventitial injuries occur in 40% of cases and are almost always fatal due to rapid exsanguination
+
+# CLINICAL ISSUES
+
+- ## Presentation
+
+
+ - ### Most common signs/symptoms
+
+
+ - No specific or sensitive signs or symptoms until hemodynamic instability ensues
+ - May have chest pain or dyspnea
+ - ### Other signs/symptoms
+
+
+ - Acute coarctation syndrome rare
+ - Upper extremity hypertension
+ - Decreased femoral pulses
+ - **Urgent diagnosis needed**, as 50% expire within 24 hours if untreated
+ - Multiple associated injuries: Diaphragm rupture, lung contusion, rib fracture (1st rib), sternal fracture head injury, injury to heart, spleen, and liver
+- ## Demographics
+
+
+ - ### Epidemiology
+
+
+ - Cause of death in 20% of high-speed MVCs
+- ## Natural History & Prognosis
+
+
+ - 80-90% mortality
+ - **85% die at site of trauma**, most often from MVC
+ - Survival depends on time from injury to intervention
+ - 2% long-term survival
+- ## Treatment
+
+
+ - Surgical repair
+ - For aortic root, ascending aorta, and arch
+ - Delayed repair may be acceptable in many cases
+ - Other injuries increase mortality of immediate repair
+ - 70-85% surgical survival quoted (up to 20% surgical mortality)
+ - Paraplegia in 10%; directly related to cross-clamp time
+ - Lower rates of paraplegia with techniques that integrate perfusion distal to clamped aorta
+ - β-adrenergic blocking agents decrease wall stress
+ - Endovascular stent graft repair
+ - Typically for isthmus, descending thoracic aorta, or abdominal aorta
+ - Less invasive than surgical repair
+ - Feasible in patients with multiple comorbid injuries
+ - Complete pseudoaneurysm resolution reported at 3 months
+ - Technical success in excluding tear approaches 100%
+ - Lower operative times, blood loss, paraplegia, and mortality compared with open surgical repair
+ - Require regular imaging follow-up for evaluation of complications
+ - Isolated injuries to intima (10%) may require no treatment and have been shown to resolve
+ - Limited data on optimal management
+
+# DIAGNOSTIC CHECKLIST
+
+- ## Consider
+
+
+ - Careful evaluation of chest radiograph in trauma for indirect signs of aortic transection
+- ## Image Interpretation Pearls
+
+
+ - Consider chronic pseudoaneurysm in any patient with vascular calcification at aorticopulmonary window
+
+ 4294f10e-00f0-482a-ac7d-e828f0b2b5bd
+
+## References
+
+# Selected References
+
+1. [Kapoor H et al: Minimal aortic injury: mechanisms, imaging manifestations, natural history, and management. Radiographics. 40(7):1834-47, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33006921%5Bpmid%5D)
+1. [Nagpal P et al: Advances in imaging and management trends of traumatic aortic injuries. Cardiovasc Intervent Radiol. 40(5):643-54, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28078377%5Bpmid%5D)
+1. [Cullen EL et al: Traumatic aortic injury: CT findings, mimics, and therapeutic options. Cardiovasc Diagn Ther. 4(3):238-44, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25009793%5Bpmid%5D)
+1. [Kaiser ML et al: Risk factors for traumatic injury findings on thoracic computed tomography among patients with blunt trauma having a normal chest radiograph. Arch Surg. 146(4):459-63, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21502456%5Bpmid%5D)
+1. [Aladham F et al: Traumatic aortic injury: computerized tomographic findings at presentation and after conservative therapy. J Comput Assist Tomogr. 34(3):388-94, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20498542%5Bpmid%5D)
+1. [Berger FH et al: Acute aortic syndrome and blunt traumatic aortic injury: pictorial review of MDCT imaging. Eur J Radiol. 74(1):24-39, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19665857%5Bpmid%5D)
+1. [Kwolek CJ et al: Current management of traumatic thoracic aortic injury. Semin Vasc Surg. 23(4):215-20, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=21194638%5Bpmid%5D)
+1. [Morgan TA et al: Acute traumatic aortic injuries: posttherapy multidetector CT findings. Radiographics. 30(4):851-67, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20219840%5Bpmid%5D)
+1. [Rojas CA et al: Mediastinal hematomas: aortic injury and beyond. J Comput Assist Tomogr. 33(2):218-24, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19346849%5Bpmid%5D)
+1. [Steenburg SD et al: Acute traumatic aortic injury: imaging evaluation and management. Radiology. 248(3):748-62, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18710974%5Bpmid%5D)
+1. [Anakwe RE: Traumatic aortic transection. Eur J Emerg Med. 12(3):133-5, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15891447%5Bpmid%5D)
+1. [Pacini D et al: Traumatic rupture of the thoracic aorta: ten years of delayed management. J Thorac Cardiovasc Surg. 129(4):880-4, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15821658%5Bpmid%5D)
+1. [Takahashi K et al: Multidetector CT of the thoracic aorta. Int J Cardiovasc Imaging. 21(1):141-53, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15915947%5Bpmid%5D)
+1. [Alkadhi H et al: Vascular emergencies of the thorax after blunt and iatrogenic trauma: multi-detector row CT and three-dimensional imaging. Radiographics. 24(5):1239-55, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15371605%5Bpmid%5D)
+1. [Kondo N et al: Surgical repair for chronic traumatic thoracic aneurysm after 12-year follow-up. Jpn J Thorac Cardiovasc Surg. 52(12):586-8, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15651408%5Bpmid%5D)
+1. [Neuhauser B et al: Stent-graft repair for acute traumatic thoracic aortic rupture. Am Surg. 70(12):1039-44, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15663041%5Bpmid%5D)
+1. [Stamenkovic SA et al: Emergency endovascular stent grafting of a traumatic thoracic aortic dissection. Int J Clin Pract. 58(12):1165-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15646416%5Bpmid%5D)
+1. [Wong H et al: Periaortic hematoma at diaphragmatic crura at helical CT: sign of blunt aortic injury in patients with mediastinal hematoma. Radiology. 231(1):185-9, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14990823%5Bpmid%5D)
+1. [Czermak BV et al: Placement of endovascular stent-grafts for emergency treatment of acute disease of the descending thoracic aorta. AJR Am J Roentgenol. 179(2):337-45, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12130430%5Bpmid%5D)
+1. [Richens D et al: The mechanism of injury in blunt traumatic rupture of the aorta. Eur J Cardiothorac Surg. 21(2):288-93, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11825737%5Bpmid%5D)
+1. [Thompson CS et al: Acute traumatic rupture of the thoracic aorta treated with endoluminal stent grafts. J Trauma. 52(6):1173-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12045649%5Bpmid%5D)
+1. [Dyer DS et al: Thoracic aortic injury: how predictive is mechanism and is chest computed tomography a reliable screening tool? A prospective study of 1,561 patients. J Trauma. 48(4):673-82; discussion 682-3, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10780601%5Bpmid%5D)
+1. [Fishman JE: Imaging of blunt aortic and great vessel trauma. J Thorac Imaging. 15(2):97-103, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10798628%5Bpmid%5D)
+1. [Dyer DS et al: Can chest CT be used to exclude aortic injury? Radiology. 213(1):195-202, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10540662%5Bpmid%5D)
+1. [Fishman JE et al: Direct versus indirect signs of traumatic aortic injury revealed by helical CT: performance characteristics and interobserver agreement. AJR Am J Roentgenol. 172(4):1027-31, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10587141%5Bpmid%5D)
+1. [Mirvis SE et al: Use of spiral computed tomography for the assessment of blunt trauma patients with potential aortic injury. J Trauma. 45(5):922-30, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9820704%5Bpmid%5D)
+1. [Patel NH et al: Imaging of acute thoracic aortic injury due to blunt trauma: a review. Radiology. 209(2):335-48, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9807557%5Bpmid%5D)
+1. [Ahrar K et al: Angiography in blunt thoracic aortic injury. J Trauma. 42(4):665-9, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9137255%5Bpmid%5D)
+1. [Mirvis SE et al: Traumatic aortic injury: diagnosis with contrast-enhanced thoracic CT--five-year experience at a major trauma center. Radiology. 200(2):413-22, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8685335%5Bpmid%5D)
+1. [Gavant ML et al: Blunt traumatic aortic rupture: detection with helical CT of the chest. Radiology. 197(1):125-33, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7568809%5Bpmid%5D)
+
+## Anatomy
+
+### Aortic Arch and Great Vessels
+Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '2cd036e9-d3d3-4b44-93f1-c0f8bf83ac5e', 'value': None}, {'key': 'd69a0534-8989-4b83-b19d-c3b640f22e53', 'value': 'Megan R. Saettele, MD'}, {'key': '2e78a4ff-418e-46f9-93e4-c6d95d960e35', 'value': 'Brandt C. Wible, MD'}], 'caseVersionId': '81c9c9a6-7b8a-498f-9b52-ae8887874927', 'description': 'Post-contrast CT images (#1-2) demonstrate a contour abnormality involving the proximal descending aorta with extravasation of contrast (arrows) and heterogeneous mediastinal soft tissue density consistent with blood products (open arrow). The patient was taken to the angiography suite for further evaluation. A 45-degree LAO projection aortogram with a pigtail catheter within the ascending aorta (#3) demonstrates focal irregularity involving the isthmus segment of the aorta as well as focal contrast extravasation (arrow), which both correlate with the site of suspected abnormality on CT scan. An additional aortographic image (#4) further confirms contrast extravasation (arrow). This lesion was not felt to be amenable to repair in the angiography suite, as the patient had other injuries requiring surgical evaluation. Aortic stent-graft placement was performed by vascular surgery in the operating room.\n\nComment: The vast majority of aortic injuries (~90%) involve the isthmus followed by the arch at the brachiocephalic trunk, often with avulsion of the latter. These injuries classically present as mediastinal widening on chest X-rays. Contrast-enhanced CT with thin sections allowing multiplanar reformatting is the test of choice for excluding aortic injury. Findings confirming aortic trauma include pseudoaneurysms, intramural hematoma, intraluminal filling defects, and active contrast extravasation. Angiography has been largely replaced by CT in the evaluation of aortic trauma, unless endovascular intervention is planned.', 'history': 'Patient presented to the emergency room status post high-speed automobile accident.', 'imagePoolId': '8b308ca9-b576-4f9e-9fac-ca5578c2e6ef', 'name': 'Traumatic injury to descending aorta', 'teachingPoint': None, 'demographics': '25 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': 'e987d3d3-1206-48d6-824b-3347c2968855', 'value': 'Michael P. Federle, MD, FACR'}, {'key': '3d84d682-9451-4b02-99b2-e34970a5b440', 'value': 'Michael P. Federle, MD, FACR'}], 'caseVersionId': 'd8eb5368-9cf3-43d5-92a4-aab723c89f36', 'description': 'A series of axial (#1-8), coronal (#9-12), sagittal (#13-14), and curved planar (#15-16) reformations of a CECT study show multiple traumatic injuries, including a traumatic laceration of the aorta, with a mucosal flap (curved arrows, #1-2, 10-11, 15-16) and adjacent mediastinal hematoma, bilateral chest tubes in the pleural space (open arrow, #1-3), subcutaneous air in the left chest wall, extensive consolidation of the right lower lobe (black arrow, #1, 3, 12-13), and extensive rib fractures, some with displacement (curved black arrows, #5-6). The liver seems to be displaced into the right hemithorax and contains several irregular, linear and rounded foci of lower attenuation (black arrow, #7-8) indicative of hepatic laceration. The liver lies in a dependent position within the right hemithorax, almost "touching" the posterior ribs and spine.\n\nCoronal and sagittal reformations confirm the displacement of the right lobe of the liver into the right hemithorax. There is a subtle acute angulation or indentation of the surface of the liver (white arrow, #11, 13-14) at the point where the liver bulges upward into the thorax.\n\nComment: As with most cases of traumatic rupture of the diaphragm, there are numerous other injuries present in this case, which may distract or impede the radiologist from accurate diagnosis. Right-sided ruptures of the diaphragm are less common, at least clinically, than those on the left, presumably because of the protective effect of the liver. This patient had immediate aortic stent repair of the aortic laceration and subsequent repair of the right diaphragmatic laceration.', 'history': 'Seat-belted victim of high-speed motor vehicle crash.', 'imagePoolId': '1ea41479-e718-45f6-98f3-93494d6c4df2', 'name': 'Right side, on multiplanar CT', 'teachingPoint': None, 'demographics': '42 Years old male'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*AP chest radiograph in a young man struck by a car shows widening of the superior mediastinum , a left apical cap , a right tracheal/endotracheal and enteric tube deviation , thick paratracheal stripes, and loss of the aortic arch and the AP window .*
+
+
+*AP chest radiograph in a young man struck by a car shows widening of the superior mediastinum , a left apical cap , a right tracheal/endotracheal and enteric tube deviation , thick paratracheal stripes, and loss of the aortic arch and the AP window .*
+
+
+*Axial CTA in the same patient shows active contrast extravasation from the ruptured descending aorta and a large mediastinal hematoma that produces mass effect on the esophagus, airways, and pulmonary arteries.*
+
+
+*Axial CTA in a 23-year-old man with a stab wound to the anterior chest shows mediastinal hemorrhage and laceration of the anteromedial ascending aorta with adjacent intramural hematoma .*
+
+
+*Sagittal oblique aortogram following blunt chest trauma shows a large pseudoaneurysm at the aortic isthmus. CTA has largely replaced conventional angiography for the diagnosis of traumatic aortic injury (TAI) but still plays an important role in TAI treatment with endovascular stent graft placement.*
+
+
+*Sagittal CECT in a patient involved in a motor vehicle collision shows a transverse segmental tear at the aortic isthmus without a surrounding mediastinal hematoma, consistent with MAI. The patient had no additional chest injuries, was treated conservatively, and was followed annually with CT.*
+
+
+*Axial CTA in the same patient 5 years following trauma shows calcification and thrombus within a chronic posttraumatic aortic pseudoaneurysm.*
+
+
+*Axial (left) and sagittal (right) CTA in a patient with blunt chest trauma show a minimal aortic injury manifesting with a focal intimal flap in the descending aorta without mediastinal hemorrhage.*
+
+
+*Axial (left) and sagittal (right) CECT in the same patient 9 days later show resolution of the aortic abnormalities. Although minimal aortic injuries are often observed and usually resolve, there is limited data on their optimal management.*
+
+
+*Axial CTA of the chest in a 60-year-old unrestrained man following a high-speed motor vehicle collision shows a linear tear from the anterior aspect of the descending aorta at the level of the diaphragm . The patient was hemodynamically unstable and was brought to the operating room.*
+
+
+*AP DSA performed intraoperatively (left) in the same patient shows the aortic injury , which was successfully treated with an endovascular stent graft (right).*
+
+
+### Additional Images
+
+
+*AP radiograph (left) shows abnormal soft tissue in the superior mediastinum that obscures the normal aortic contour and displaces the trachea to the right. Additionally, there is widening of the paravertebral stripe . Coronal CT shows a traumatic aortic pseudoaneurysm at the aortic isthmus with surrounding mediastinal and periaortic hematoma.*
+
+
+*Coronal CECT shows the aortic pseudoaneurysm with surrounding mediastinal hematoma that prevents the aortic arch from being visualized on radiograph.*
+
+
+*Coronal MIP shows the paraaortic hematoma extending along the entire course of the thoracic aorta.*
+
+
+*AP chest radiograph shows a widened mediastinum, enlargement of the aortic arch , and tracheal deviation to the right secondary to traumatic aortic transection.*
+
+
+*Axial CECT in the same patient shows a pseudoaneurysm at the site of aortic injury in the proximal descending aorta with surrounding mediastinal hemorrhage .*
+
+
+*Sagittal CTA in the same patient shows the aortic tear contained by a pseudoaneurysm. Note the proximity of this injury to the ligamentum arteriosum .*
+
+
+*Coronal CECT in a patient with TAI (not shown) shows the ligamentum arteriosum and mediastinal hemorrhage .*
+
+
+*Coronal CECT at a more posterior level demonstrates the aortic tear .*
+
+
+*Sagittal oblique DSA shows a contained transection of the descending aorta . An injury of this degree of severity is at a very high risk for imminent rupture. (Courtesy J. Caridi, MD.)*
+
+
+*Axial CTA shows an aortic pseudoaneurysm resulting from a concentric aortic tear with a large surrounding mediastinal hematoma . The CTA findings are diagnostic, and there is no need for DSA.*
+
+
+*Sagittal oblique DSA shows a tear of both the ascending aorta , near the left main coronary artery origin, and the proximal descending aorta . (Courtesy J. Caridi, MD.)*
+
diff --git a/results.json b/results.json
index e49f4f6..2d3a61f 100644
--- a/results.json
+++ b/results.json
@@ -1,116 +1,550 @@
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- "title": "Multiinfarct Dementia",
- "docid": "3823c4d4-5e98-46da-a717-892fef54b382",
+ "path": "docs_md/articles/double-aortic-arch_0b68477f-f05a-4e4c-a1a8-02fe29c292db.md",
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{
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- "docid": "0109f4c0-c84a-4d85-97cb-afe437b9cc43",
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\ No newline at end of file