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---
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title: "Aberrant Right Subclavian Artery"
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docid: "a20702fe-5409-44a5-a804-cb071023bade"
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authors:
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- key: "770e1d77-2287-436e-910b-48232afc7842"
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value: "Prabhakar Rajiah, MBBS, MD, FACR, FRCR, FACC, FAHA, FSCCT"
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- key: "e915766e-8102-46e4-a33e-c83f8ae12f29"
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value: "Harold Goerne, MD"
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breadcrumbs:
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name: "Cardiac"
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slug: "cardiac"
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name: "Diagnosis"
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name: "Aorta"
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slug: "aorta"
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treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
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name: "Aberrant Right Subclavian Artery"
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slug: "aberrant-right-subclavian-artery"
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category: "Cardiac"
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documentVersionId: "69195035-5819-4996-b27d-b107c62d7c60"
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imageCount: 12
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lastUpdated: "01/28/25"
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pageDescription: "Aberrant Right Subclavian Artery"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Aberrant Right Subclavian Artery"
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pageTitle: "Aberrant Right Subclavian Artery | STATdx"
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||||
enhancedTitle: "Aberrant Right Subclavian Artery"
|
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type: "DX"
|
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references: true
|
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breadcrumbs:
|
||||
- "Cardiac"
|
||||
- "Diagnosis"
|
||||
- "Aorta"
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||||
- "Aberrant Right Subclavian Artery"
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---
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# KEY FACTS
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- ## Terminology
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- Aberrant right subclavian artery (ARSA) as last branch of left-sided aortic arch, isthmus, or proximal descending aorta
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- Course: Retroesophageal 80%; intertracheoesophageal 15%; pretracheal 5%
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- ## Imaging
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- Barium esophagogram shows persistent esophageal narrowing at level of extrinsic compression with oblique course
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- CTA or MRA help to understand origin and course of supraaortic trunks
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- No vascular ring formed by left aortic arch and ARSA
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- Loose vascular ring if ARSA originates from Kommerell diverticulum
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- Complete vascular ring if ARSA with circumflex right descending thoracic aorta with right ductus
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- ## Top Differential Diagnoses
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- Major aortopulmonary collateral arteries (MAPCAs)
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- Right arch with aberrant left subclavian artery
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- Retroesophageal diverticulum
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- ## Pathology
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- Regression of right 4th arch between right subclavian and right common carotid arteries, including right ductus arteriosus
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- ## Clinical Issues
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- Most common congenital aortic arch abnormality (0.5-2.0%)
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- 90-95% asymptomatic; incidental imaging finding
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- Adults: Dysphagia (lusoria), dyspnea, back pain
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- Infants: Cough, stridor, aspiration pneumonia
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- Surgery for symptoms, aneurysm, or large diverticulum
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# TERMINOLOGY
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- ## Abbreviations
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- Aberrant right subclavian artery (ARSA)
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- ## Synonyms
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- Lusoria artery (arteria lusoria)
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- Incomplete vascular ring
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- ## Definitions
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- Aberrant origin of right subclavian artery as last branch of left-sided aortic arch or proximal descending aorta
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- Normally RSA originates from brachiocephalic artery
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- ARSA as last branch of left-sided aortic arch or proximal descending aorta
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- Retroesophageal/retrotracheal course of ARSA
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- ### Location
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- ARSA takeoff from distal aortic arch, isthmus, or proximal descending aorta
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- Course: Retroesophageal 80%; intertracheoesophageal 15%; pretracheal 5%
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- ### Size
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- Usually normal vessel caliber
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- Dilated proximal segment in Kommerell diverticulum
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- Aneurysm may be present
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- ### Morphology
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- No vascular ring formed by left aortic arch and ARSA
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- Trachea and esophagus are in middle between aortic arch and ARSA
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- Loose vascular ring if ARSA originates from Kommerell diverticulum
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- Right ductus/ligamentum arteriosum completes ring
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- Ductus arising from junction between diverticulum and normal-sized subclavian artery
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- Seen in 15-30% of ARSA
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- Complete vascular ring if ARSA with circumflex right descending thoracic aorta and right ductus
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- Arch itself crosses midline, posterior to esophagus
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- Right ductus extends from descending aorta to right pulmonary artery, completing vascular ring
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- ## Radiographic Findings
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- Chest x-ray may show enlargement of superior mediastinum
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- Barium esophagogram shows persistent esophageal narrowing at level of extrinsic compression with oblique course
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- ## CT Findings
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- ### CTA
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- Excellent modality to demonstrate aortic arch configuration and branching pattern
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- MPR, MIP, and VRT reconstructions help to understand origin and course of supraaortic trunks
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- Evaluates esophageal and tracheal compression
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- NECT: May be seen incidentally
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- 4D CT: May show dynamic esophageal compression during cardiac cycle due to distension of ARSA during systole
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- ## MR Findings
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- ### MRA
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- Arch vessel branching pattern is (from right to left): Right common carotid artery; left common carotid artery; left subclavian artery; right subclavian artery (RSA)
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- Course: Obliquely from caudal left to cranial right
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- Aneurysm of ARSA: 1.5x d of distal subclavian artery
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- Left ductus may be seen; no vascular ring
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- Since there is vasculature on only 3 sides of trachea and esophagus
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- Posterior esophageal compression in 10%
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- Usually in 4th or 5th decade
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- Stretching, sclerosis/calcification, aneurysm of ARSA
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- Complete vascular ring may be seen if
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- Kommerell diverticulum: Dilation of origin of ARSA
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- Diameter of orifice of diverticulum and distance from furthest aortic wall to end of diverticulum measured
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- Circumflex right descending thoracic aorta with arch behind esophagus
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- ### SSFP cine
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- May show dynamic esophageal compression during cardiac cycle due to vessel distension during systole
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- ## Imaging Recommendations
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- ### Best imaging tool
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- CTA or MRA
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- ### Protocol advice
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- ECG gating is not necessary if only evaluation of vascular ring is desired
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- High-pitch helical mode of dual source CT scanner (Flash) mode, provide anatomic information
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# DIFFERENTIAL DIAGNOSIS
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- ## Major Aortopulmonary Collateral Arteries
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- Collateral vessels arising from descending aorta that end in lungs in patients with pulmonary artery atresia
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- [Right Arch With Aberrant Left Subclavian Artery](/document/right-aortic-arch/5f186c96-4cc3-453e-840d-12ebfad13115)
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- Mirror image of left arch with ARSA
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- Caused by persistent right 4th arch and regression of left 4th arch in between left common carotid and left subclavian arteries
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- Aberrant left subclavian artery originates as last branch from right aortic arch and courses behind esophagus to reach left
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- 90% have left ductus vascular ring
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- 2nd most common cause of complete vascular ring
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- ## Retroesophageal Diverticulum
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- Outpouching at origin of aberrant left subclavian artery in right aortic arch; seen in 60% of these cases
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Embryologic insult during formation of aortic arches
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- Right 4th arch regresses between RSA and right common carotid artery, including ductus arteriosus
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- Distal right dorsal aorta forms proximal RSA
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- Distal portion from 7th right intersegmental artery
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- Kommerell diverticulum: Remnant of dorsal aortic arch
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- Persistence of right 6th arch component that forms right ductus arteriosus
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- Left circumflex aorta
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- Regression of right 4th arch between right common carotid and right subclavian arteries
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- Persistence of right 6th arch component that forms ductus
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- Right-sided descending aorta
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- Distal left dorsal aorta forms definitive distal aortic arch
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- ### Associated abnormalities
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- ARSA is present in 35% of patients with Down syndrome
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- Kommerell diverticulum: 14.9%
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- Aneurysm of distal RSCA: 12.8%
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- Others: Coarctation; patent ductus arteriosus; ventricular septal defect; truncus bicaroticus; type B interrupted arch
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- ## Staging, Grading, & Classification
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- Retroesophageal course: 80%
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- Interesophageotracheal course: 15%
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- Pretracheal course: 5%
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- 90-95% asymptomatic; usually incidental finding in imaging studies
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- ### Other signs/symptoms
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- Adults: Dysphagia (lusoria), dyspnea, back pain, arm claudication, abnormal chest x-ray
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- Infants: Cough, stridor, occasionally aspiration pneumonia
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- Rare: Ruptured diverticulum; dissection
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- ## Demographics
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- Age: Any; mean at detection 41.8 ± 26.6 years
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- Sex: F:M = 3:1
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- Epidemiology
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- Most common congenital aortic arch abnormality
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- Prevalence 0.5-2.0% of population
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- ## Natural History & Prognosis
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- Caution required during esophageal surgeries
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- Caution required in thyroid and parathyroid surgeries
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- Right inferior laryngeal nerve passes directly from vagus nerve at level of larynx to neck
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- ## Treatment
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- Surgery for symptoms, aneurysm, or large diverticulum
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- Diverticulum orifice > 3-cm or > 5-cm depth between wall adjacent to trachea and opposite aortic wall
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6f7d98b5-f858-4f63-8153-640ab0b652fd
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## References
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# Selected References
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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## Images
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### Selected Images
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*CTA cinematic rendering reconstruction shows 5 branches from the aortic arch. From the proximal to distal, the branches are: Right common carotid artery <img src='img/arrows/WS.png'/>, left internal carotid artery <img src='img/arrows/CO.png'/>, left external carotid artery <img src='img/arrows/CC.png'/>, left subclavian artery <img src='img/arrows/WO.png'/>, and aberrant right subclavian artery (ARSA) <img src='img/arrows/WC.png'/>. Mild aortic coarctation <img src='img/arrows/CS.png'/> is also noted.*
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*CTA cinematic rendering reconstruction shows 5 branches from the aortic arch. From the proximal to distal, the branches are: Right common carotid artery <img src='img/arrows/WS.png'/>, left internal carotid artery <img src='img/arrows/CO.png'/>, left external carotid artery <img src='img/arrows/CC.png'/>, left subclavian artery <img src='img/arrows/WO.png'/>, and aberrant right subclavian artery (ARSA) <img src='img/arrows/WC.png'/>. Mild aortic coarctation <img src='img/arrows/CS.png'/> is also noted.*
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*CTA cinematic rendering reconstruction shows ARSA <img src='img/arrows/WS.png'/> that originates as the last branch from the aortic arch and courses behind the esophagus to reach the right.*
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*Coronal oblique MIP CTA shows a prominent Kommerell diverticulum <img src='img/arrows/WS.png'/>, which is a dilatation of a proximal ARSA <img src='img/arrows/WO.png'/> that originates in the proximal descending aorta <img src='img/arrows/WC.png'/>.*
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*Axial oblique MIP reconstruction from a CTA shows an ARSA <img src='img/arrows/WS.png'/> with retroesophageal course causing esophageal compression <img src='img/arrows/WO.png'/> between aortic arch, trachea <img src='img/arrows/CS.png'/>, and ARSA.*
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### Additional Images
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*Axial oblique MIP reconstruction from a CTA shows an ARSA <img src='img/arrows/WS.png'/> that causes esophageal compression <img src='img/arrows/WO.png'/>.*
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*3D reconstruction of the airways and lungs from a CT in the same patient shows severe esophageal luminal narrowing <img src='img/arrows/WS.png'/> from an ARSA.*
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*MRA 3D posterior oblique view shows 6 branches from the aortic arch. From proximal to distal the branches are: A common trunk <img src='img/arrows/WS.png'/> dividing into right common carotid artery <img src='img/arrows/BS.png'/> and right vertebral artery <img src='img/arrows/BO.png'/>, left internal carotid artery <img src='img/arrows/CC.png'/>, left external carotid artery <img src='img/arrows/BC.png'/>, left vertebral artery <img src='img/arrows/WO.png'/>, left subclavian artery <img src='img/arrows/WC.png'/>, and ARSA <img src='img/arrows/CS.png'/>.*
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*Sagittal T2-weighted MR of the cervical spine shows a round structure between the spine <img src='img/arrows/WO.png'/> and esophagus <img src='img/arrows/CO.png'/>, consistent with an aberrant right subclavian artery <img src='img/arrows/WS.png'/> causing esophageal compression.*
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*Coronal MIP reconstruction from a MRA shows an ARSA <img src='img/arrows/WS.png'/> and a normal left subclavian artery <img src='img/arrows/WO.png'/>, both originating at the same level in the distal aortic arch <img src='img/arrows/CO.png'/>.*
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*CTA cinematic rendering reconstruction shows a right-sided aortic arch <img src='img/arrows/CO.png'/> with mirror-image branching <img src='img/arrows/WS.png'/> and aberrant left subclavian artery <img src='img/arrows/WO.png'/>, which originates as the last branch from the arch.*
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*Coronal MIP CTA reconstruction in a 56-year-old man shows an ARSA <img src='img/arrows/CS.png'/> originating from the proximal descending thoracic aorta <img src='img/arrows/WO.png'/> and extending towards the right.*
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*Axial MIP CTA reconstruction in a 56-year-old man shows an ARSA <img src='img/arrows/CS.png'/> originating from the proximal descending thoracic aorta <img src='img/arrows/WO.png'/> and extending towards the right, with compression of the esophagus <img src='img/arrows/WS.png'/>.*
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@@ -0,0 +1,367 @@
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---
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||||
title: "Aortic Aneurysm: Rupture"
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docid: "e64e6a27-0c8d-4b4f-8ca5-d2e65c9f2e9a"
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breadcrumbs:
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||||
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|
||||
- "Aortic Aneurysm: Rupture"
|
||||
---
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||||
# KEY FACTS
|
||||
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||||
- ## Terminology
|
||||
|
||||
|
||||
- Dilated aorta with disruption through all 3 layers of aortic wall leading to extravasation of blood into surrounding structures
|
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- 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
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- ## Imaging
|
||||
|
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|
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- Risk of rupture or dissection often linked to aneurysm size, morphology, and rate of growth
|
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- Ascending aortic aneurysms (AAs), asymptomatic
|
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- In patients with sporadic root and ascending AAs, repair considered with aneurysm size ≥ 5 cm
|
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- In patients with genetic aortopathy, repair considered if AA diameter is ≥ 4 cm
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- Descending thoracic aorta, asymptomatic
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- In patients with intact descending thoracic aneurysm, repair recommended if diameter ≥ 5.5 cm
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- In patients with high risk of rupture, repair recommended if diameter ≥ 5 cm
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- Abdominal aortic aneurysm (AAA), asymptomatic
|
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- Elective repair with AAA diameter of ≥ 5.5 cm and ≥ 5 cm in men and women, respectively
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- Symptomatic patients should undergo repair regardless of size
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- Depending on rate of growth, repair recommended with
|
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- Sporadic thoracic aneurysms and growth rate ≥ 0.5 cm in 1 year or ≥ 0.3 cm per year in 2 consecutive years
|
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- 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 <img src='img/arrows/CC.png'/> with a partially loculated right pleural effusion <img src='img/arrows/CO.png'/>.*
|
||||
|
||||
|
||||
*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 <img src='img/arrows/CC.png'/> with a partially loculated right pleural effusion <img src='img/arrows/CO.png'/>.*
|
||||
|
||||
|
||||
*CTA in the same patient after he traveled to the USA shows rupture of a large thoracoabdominal aortic aneurysm <img src='img/arrows/CC.png'/> with right hemothorax <img src='img/arrows/CO.png'/> and blood in the mediastinum <img src='img/arrows/CS.png'/>.*
|
||||
|
||||
|
||||
*Delayed phase from the CTA in the same patient better shows complex loculated fluid collections in right pleural space <img src='img/arrows/CO.png'/> and mediastinum <img src='img/arrows/CS.png'/> due to blood products. The aneurysm <img src='img/arrows/CC.png'/> 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 <img src='img/arrows/CS.png'/>. The left kidney is infarcted <img src='img/arrows/CC.png'/>. DWI image (R) 1 day after repair shows a large PCA territory infarct <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
*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 <img src='img/arrows/CS.png'/>, 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 <img src='img/arrows/CO.png'/> and false <img src='img/arrows/CS.png'/> lumens. The false lumen has ruptured <img src='img/arrows/CC.png'/>, leading to extensive mediastinal hematoma <img src='img/arrows/WC.png'/> and hemothorax <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
*Axial CT in an 83-year-old man with severe chest pain shows a thoracic aortic aneurysm with mild irregularity of the right wall <img src='img/arrows/CS.png'/> with surrounding mediastinal hematoma <img src='img/arrows/CO.png'/> and large right hemothorax <img src='img/arrows/CC.png'/>. 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 <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> with surrounding soft tissue <img src='img/arrows/WO.png'/>, 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 <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/WS.png'/> and post CABG changes <img src='img/arrows/WO.png'/>. The ascending aorta is aneurysmal <img src='img/arrows/WC.png'/> with dissection involving both the ascending <img src='img/arrows/BS.png'/> and descending <img src='img/arrows/BO.png'/> thoracic aorta.*
|
||||
|
||||
|
||||
*Inferior and oblique CTA in the same patient shows there is a large mediastinal hematoma <img src='img/arrows/WS.png'/> communicating with the aneurysmal ascending thoracic aorta <img src='img/arrows/WO.png'/> via a focal discontinuity, and fistulation with the right ventricular outflow tract <img src='img/arrows/WC.png'/>, compatible with rupture.*
|
||||
|
||||
|
||||
*NECT in a man in his 80's who presented with chest pain, AMS, and shock shows an aneurysmal ascending aorta <img src='img/arrows/WS.png'/> with a large, hyperattenuating intramural hematoma <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
*Slightly inferior NECT following contrast administration in the same patient shows an ascending thoracic aortic aneurysm <img src='img/arrows/WS.png'/> and mural thickening <img src='img/arrows/WO.png'/> representing intramural hematoma as well as large volume hemopericardium <img src='img/arrows/WC.png'/>, compatible with rupture into the pericardial space.*
|
||||
|
||||
File diff suppressed because one or more lines are too long
+136
@@ -0,0 +1,136 @@
|
||||
---
|
||||
title: "Aortic Intramural Abnormality"
|
||||
docid: "75d7b37f-bc37-493b-8961-8b2a9001fb94"
|
||||
authors:
|
||||
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|
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|
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|
||||
pageDescription: "Aortic Intramural Abnormality"
|
||||
pageKeywords: "Cardiac, Differential Diagnosis, Aortic Intramural Abnormality"
|
||||
pageTitle: "Aortic Intramural Abnormality | STATdx"
|
||||
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|
||||
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|
||||
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|
||||
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|
||||
- "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 <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
**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 <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
**Aortic Dissection**
|
||||
*Axial CECT shows a severely displaced dissection flap, compressing the true lumen and occluding the SMA <img src='img/arrows/WO.png'/>. Note true lumen <img src='img/arrows/WS.png'/> and false lumen <img src='img/arrows/WC.png'/>. This was treated with fenestration.*
|
||||
|
||||
|
||||
**Aortic Dissection**
|
||||
*Axial CECT shows a dissection flap <img src='img/arrows/WC.png'/> in the descending aorta with a displaced intimal calcification <img src='img/arrows/WS.png'/>. The dissection did not involve the arch and was managed medically.*
|
||||
|
||||
|
||||
**Aortic Intramural Hematoma**
|
||||
*Coronal NECT shows asymmetric thickening and hyperdense <img src='img/arrows/BS.png'/> aortic wall, representing the hematoma. Note the displaced intimal calcification <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
**Aortic Intramural Hematoma**
|
||||
*Axial CECT shows hyperdense, thickened aortic wall <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WC.png'/> extending beyond the expected aortic wall <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/WS.png'/> with inner intimal calcifications. Although causing aortic narrowing, this may progress to aneurysmal dilation.*
|
||||
|
||||
|
||||
**Radiation**
|
||||
*Double oblique CECT shows dense aortic <img src='img/arrows/BS.png'/> and pulmonary artery <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
@@ -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 <img src='img/arrows/CO.png'/> and descending <img src='img/arrows/CC.png'/> aorta.*
|
||||
|
||||
|
||||
*Axial NECT in a patient with Stanford type A intramural hematoma (IMH) shows crescent-shaped wall hyperdensity at the ascending <img src='img/arrows/CO.png'/> and descending <img src='img/arrows/CC.png'/> aorta.*
|
||||
|
||||
|
||||
*Axial CT angiography shows crescentic aortic wall thickening in the ascending <img src='img/arrows/WO.png'/> and descending <img src='img/arrows/WC.png'/> aorta. Type A IMH is typically treated surgically.*
|
||||
|
||||
|
||||
*Narrow window settings can improve the visibility of the hyperattenuated, crescent-shaped ascending <img src='img/arrows/CO.png'/> and descending <img src='img/arrows/CC.png'/> aortic wall. A hypoattenuating, crescent-shaped thickened intima <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> along the descending thoracic aorta. This intensity behavior is consistent with acute hemorrhage.*
|
||||
|
||||
|
||||
*Axial SSFP MR in the same patient shows that the IMH <img src='img/arrows/BS.png'/> 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 <img src='img/arrows/CC.png'/>. Note decreased diameter <img src='img/arrows/CS.png'/> 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) <img src='img/arrows/BS.png'/> with a broad neck along the distal thoracic aorta, likely the primary cause of the IMH. Note also an ulcer-like projection (ULP) <img src='img/arrows/WC.png'/> along the aortic arch.*
|
||||
|
||||
|
||||
*Axial CECT in a patient with type B IMH <img src='img/arrows/CC.png'/> shows focal aortic projection <img src='img/arrows/CS.png'/> 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 <img src='img/arrows/CS.png'/> and new aortic dissection <img src='img/arrows/CC.png'/>. 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) <img src='img/arrows/CC.png'/>. There is no clear communication with the aortic lumen, but there is visible communication with an adjacent intercostal artery <img src='img/arrows/CS.png'/>.*
|
||||
|
||||
|
||||
*Sagittal oblique CTA in the same patient shows multilevel IBP <img src='img/arrows/CS.png'/> 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 <img src='img/arrows/WS.png'/>. CTA demonstrates the classic crescentic wall thickening <img src='img/arrows/BC.png'/>.*
|
||||
|
||||
|
||||
*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 <img src='img/arrows/WC.png'/> show a coexistent descending thoracic aortic dissection <img src='img/arrows/BS.png'/>.*
|
||||
|
||||
|
||||
*Axial chest NECT and CTA in a patient with incomplete dissection show crescentic hyperdensity <img src='img/arrows/WS.png'/> and thickening <img src='img/arrows/BC.png'/> along the ascending aorta, findings identical to those seen in IMH. Note the discrete bulging <img src='img/arrows/WO.png'/> 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 <img src='img/arrows/WS.png'/>, consistent with IMH along the ascending and descending thoracic aorta.*
|
||||
|
||||
|
||||
*Axial chest CTA in the same patient shows concentric thickening along the ascending <img src='img/arrows/BS.png'/> and descending <img src='img/arrows/WS.png'/> thoracic aorta as well as a small IBP along the descending thoracic aorta <img src='img/arrows/WC.png'/>. Note the small pericardial and bilateral pleural effusions, which are common nonspecific findings.*
|
||||
|
||||
|
||||
*Sagittal oblique CTA in the same patient shows IMH <img src='img/arrows/BS.png'/> 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 <img src='img/arrows/CS.png'/>. The pleura <img src='img/arrows/CC.png'/> 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 <img src='img/arrows/CS.png'/> with an area of more dense calcification <img src='img/arrows/CC.png'/>.*
|
||||
|
||||
|
||||
*Axial CECT shows the circumferential ascending aortic soft tissue <img src='img/arrows/CS.png'/>. 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.*
|
||||
|
||||
@@ -0,0 +1,137 @@
|
||||
---
|
||||
title: "Cardiac Calcifications"
|
||||
docid: "c53ad786-4464-4a04-a3e9-ccd286e1f8fc"
|
||||
authors:
|
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|
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|
||||
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|
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|
||||
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|
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|
||||
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|
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|
||||
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 <img src='img/arrows/WS.png'/> in a patient with LAD atherosclerosis. Note the presence of noncalcified plaque <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
**Coronary Arteries**
|
||||
*Axial oblique CECT MIP shows discrete calcifications in a linear arrangement <img src='img/arrows/WS.png'/> in a patient with LAD atherosclerosis. Note the presence of noncalcified plaque <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
**Mitral Valve Leaflets**
|
||||
*Frontal radiograph shows characteristic reversed C-shaped calcification <img src='img/arrows/BS.png'/> 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 <img src='img/arrows/WS.png'/> presumed to be due to rheumatic heart disease. Note the enlarged left atrium and left atrial calcifications <img src='img/arrows/WO.png'/>. The patient also has aortic stenosis and calcifications <img src='img/arrows/WC.png'/>.*
|
||||
|
||||
|
||||
**Aortic Valve**
|
||||
*Double oblique CECT MIP shows dense calcifications of the aortic valve cusps <img src='img/arrows/WS.png'/> in a patient with severe aortic stenosis. Calcium burden correlates with severity of stenosis.*
|
||||
|
||||
|
||||
**Pericardial**
|
||||
*Axial NECT shows pericardial calcification <img src='img/arrows/WS.png'/> at the atrioventricular grooves. Note the epicardial fat <img src='img/arrows/WO.png'/> to differentiate from coronary calcium.*
|
||||
|
||||
|
||||
**Myocardial**
|
||||
*Left ventricular outflow view shows apical calcification <img src='img/arrows/WS.png'/> and wall thinning in a patient with prior myocardial infarction. Note the epicardial fat to differentiate from pericardium <img src='img/arrows/WO.png'/>. Wall motion abnormality was present (not shown).*
|
||||
|
||||
|
||||
**Neoplastic Mass**
|
||||
*Axial NECT (left) and bright blood MR (right) show a new calcification <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
**Neoplastic Mass**
|
||||
*Lateral radiograph shows curvilinear calcification <img src='img/arrows/WO.png'/> in the left atrium in a patient with left atrial myxoma.*
|
||||
|
||||
|
||||
### Additional Images
|
||||
|
||||
|
||||
**Mitral Valve Leaflets**
|
||||
*Four-chamber CECT shows both papillary muscle <img src='img/arrows/WS.png'/> and mitral valve annular <img src='img/arrows/WO.png'/> calcification. Papillary muscle calcification is commonly seen in dialysis patients.*
|
||||
|
||||
@@ -0,0 +1,203 @@
|
||||
---
|
||||
title: "Cardiac Mass"
|
||||
docid: "fa1f894a-4619-4657-b99b-a967a3e4e871"
|
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|
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||||
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|
||||
---
|
||||
# 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 <img src='img/arrows/BS.png'/> with adjacent calcifications <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/BS.png'/> with adjacent calcifications <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> in a patient with atrial fibrillation. Atrial appendages are common locations for thrombi.*
|
||||
|
||||
|
||||
**Thrombus**
|
||||
*Filling defect <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/BS.png'/> (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 <img src='img/arrows/WS.png'/>. Note heterogeneous contrast attenuation.*
|
||||
|
||||
|
||||
**Cardiac Metastases**
|
||||
*Axial CECT shows a heterogeneous mass in right and left atrium <img src='img/arrows/WS.png'/>. Although sparing of the fossa ovalis <img src='img/arrows/BC.png'/> 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 <img src='img/arrows/WS.png'/> in the left atrium, which represented metastatic B-cell lymphoma.*
|
||||
|
||||
|
||||
**Myxoma**
|
||||
*Four-chamber bright-blood MR shows filling defect in LA <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/WS.png'/>. Note sparing of fossa ovalis <img src='img/arrows/WO.png'/> and near-complete loss of signal with fat suppression <img src='img/arrows/WC.png'/>. This is benign but may be confused with a cardiac mass.*
|
||||
|
||||
|
||||
**Sarcoma**
|
||||
*Axial CECT shows a heterogeneous, enhancing mass filling the right atrium <img src='img/arrows/WS.png'/> with extension into the pericardium and obliteration of the epicardial fat <img src='img/arrows/WC.png'/>. There was no pericardial effusion. Pathology revealed an angiosarcoma.*
|
||||
|
||||
|
||||
**Sarcoma**
|
||||
*Axial C+ MR in the same patient shows heterogeneous contrast enhancement <img src='img/arrows/WS.png'/>. The right atrium is the most common location for cardiac angiosarcoma.*
|
||||
|
||||
|
||||
**Sarcoma**
|
||||
*Axial CECT shows left posterior atrial wall thickening with a lobulated contour <img src='img/arrows/WS.png'/>. The mass has a broad attachment base. Resection demonstrated a leiomyosarcoma.*
|
||||
|
||||
|
||||
**Hemangioma**
|
||||
*Axial T2 black-blood MR shows a high-signal right atrial mass <img src='img/arrows/WS.png'/>. Note the heterogeneous enhancement following IV contrast administration. Surgical removal revealed hemangioma.*
|
||||
|
||||
|
||||
### Additional Images
|
||||
|
||||
|
||||
**Cardiac Metastases**
|
||||
*Axial CECT shows filling defect in SVC <img src='img/arrows/WS.png'/>, which was suspected to be thrombus. Multiple other filling defects were present in the right atrium (not shown). Right skin lesion <img src='img/arrows/WO.png'/> is noted, and biopsy showed large B-cell lymphoma. SVC mass was later proven to be a tumor.*
|
||||
|
||||
+370
@@ -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 <img src='img/arrows/BS.png'/>. There is elevation of the right hemidiaphragm form chronic undiagnosed right diaphragmatic hernia <img src='img/arrows/WO.png'/>. Note remote rib fractures <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*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 <img src='img/arrows/BS.png'/>. There is elevation of the right hemidiaphragm form chronic undiagnosed right diaphragmatic hernia <img src='img/arrows/WO.png'/>. Note remote rib fractures <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Lateral radiograph of the chest in the same patient shows aortic bulging with intrinsic mural calcification <img src='img/arrows/WC.png'/> at the isthmus.*
|
||||
|
||||
|
||||
*Axial chest CTA in the same patient demonstrates saccular isthmic aortic dilatation <img src='img/arrows/WS.png'/> with some mural calcifications <img src='img/arrows/WC.png'/> in continuity with the aortic lumen.*
|
||||
|
||||
|
||||
*Axial chest CTA in the same patient reveals extensive calcifications <img src='img/arrows/WC.png'/> 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 <img src='img/arrows/WO.png'/> at the aortic isthmus. Note characteristic sudden change in caliber <img src='img/arrows/WC.png'/> 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 <img src='img/arrows/WC.png'/>.*
|
||||
|
||||
|
||||
*Posterior sagittal chest 3D reformation in the same patient demonstrates the aortic pseudoaneurysm <img src='img/arrows/WO.png'/> 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 <img src='img/arrows/BC.png'/> along the inferior aspect of the aortic arch.*
|
||||
|
||||
|
||||
*Axial chest CTA in the same patient demonstrates well-marginated saccular dilatation <img src='img/arrows/WS.png'/> of the aorta at the level of the isthmus.*
|
||||
|
||||
|
||||
*Oblique sagittal reformation chest CTA in the same patient shows an isthmic pseudoaneurysm <img src='img/arrows/WO.png'/> 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) <img src='img/arrows/WC.png'/> 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 <img src='img/arrows/WS.png'/> at the level of the aortic isthmus.*
|
||||
|
||||
|
||||
*Axial CTA in the same patient shows a saccular aneurysm <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/BS.png'/> 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 <img src='img/arrows/BO.png'/>. 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 <img src='img/arrows/WO.png'/> with eccentric peripheral calcification <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Left anterior oblique aortogram in the same patient shows contrast filling the aortic pseudoaneurysm <img src='img/arrows/BS.png'/>. 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 <img src='img/arrows/WO.png'/> along the aortic isthmus with peripheral rim calcification <img src='img/arrows/WS.png'/>, 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 <img src='img/arrows/WS.png'/> to the descending aorta <img src='img/arrows/WO.png'/>. The pseudoaneurysm is thrombosed <img src='img/arrows/BO.png'/>, and there is no endoleak.*
|
||||
|
||||
|
||||
*Axial CECT of the same patient shows a thrombosed <img src='img/arrows/WC.png'/> saccular pseudoaneurysm sac that has been excluded by an endovascular aortic stent graft <img src='img/arrows/WO.png'/> used for treatment. The endovascular graft lumen is widely patent <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Axial CECT shows a peripherally calcified enhancing saccular outpouching <img src='img/arrows/WS.png'/> from the distal aortic arch with compression of the left main stem bronchus <img src='img/arrows/WO.png'/>. 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 <img src='img/arrows/BO.png'/>. The CT and angiographic features, along with the patient's history, are consistent with a posttraumatic thoracic aortic pseudoaneurysm.*
|
||||
|
||||
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@@ -0,0 +1,145 @@
|
||||
---
|
||||
title: "Dilatation of Thoracic Aorta"
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||||
docid: "3b177a9c-04f3-4d15-9d18-decc5c236e7e"
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|
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pageTitle: "Dilatation of Thoracic Aorta | STATdx"
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|
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breadcrumbs:
|
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- "Chest"
|
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|
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- "Thoracic Aorta"
|
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|
||||
- "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 <img src='img/arrows/CS.png'/> and displacement of the intrathoracic trachea to the right <img src='img/arrows/CC.png'/>. The ascending aorta <img src='img/arrows/CO.png'/> 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 <img src='img/arrows/CS.png'/> and displacement of the intrathoracic trachea to the right <img src='img/arrows/CC.png'/>. The ascending aorta <img src='img/arrows/CO.png'/> is visible overlying the right hilum.*
|
||||
|
||||
|
||||
**Atherosclerotic Aneurysm**
|
||||
*Axial CECT of the same patient shows aneurysm of the descending thoracic aorta <img src='img/arrows/CS.png'/> and calcified <img src='img/arrows/CC.png'/> 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 <img src='img/arrows/CS.png'/> of the aortic arch. Acute angles of the aneurysm <img src='img/arrows/CC.png'/> 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 <img src='img/arrows/CO.png'/> representing postsurgical material <img src='img/arrows/CC.png'/> 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 <img src='img/arrows/CS.png'/>. The intrathoracic trachea is displaced to the right <img src='img/arrows/CC.png'/>.*
|
||||
|
||||
|
||||
**Postoperative Pseudoaneurysm**
|
||||
*Coronal reformatted CECT of the same patient shows the descending thoracic aortic stent <img src='img/arrows/CC.png'/> complicated by large, complex contrast outpouchings <img src='img/arrows/CS.png'/>, consistent with postoperative pseudoaneurysms.*
|
||||
|
||||
|
||||
**Aortitis**
|
||||
*Axial CECT of a patient with sepsis and chest pain shows aneurysmal dilation of the proximal descending thoracic aorta <img src='img/arrows/CS.png'/> with associated diffuse aortic wall thickening and enhancement <img src='img/arrows/CC.png'/>, 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 <img src='img/arrows/CS.png'/>. A large left pleural effusion is also present. Staphylococcus and salmonella are frequent organisms causing infectious aortitis.*
|
||||
|
||||
@@ -0,0 +1,199 @@
|
||||
---
|
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title: "Dilated Aorta"
|
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||||
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|
||||
---
|
||||
# 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 <img src='img/arrows/WS.png'/> and intimal calcifications <img src='img/arrows/BS.png'/>. This patient had a diffusely dilated and tortuous aorta.*
|
||||
|
||||
|
||||
**Atherosclerotic**
|
||||
*Coronal CECT shows extravasation of contrast <img src='img/arrows/WS.png'/> from a dilated abdominal aorta. Note extravasated blood <img src='img/arrows/WC.png'/>, which can easily be detected on unenhanced CT.*
|
||||
|
||||
|
||||
**Atherosclerotic**
|
||||
*Axial CECT shows dilated abdominal aorta with extensive mural thrombus <img src='img/arrows/WC.png'/>. Calcifications <img src='img/arrows/WS.png'/> occur when the thrombus is chronic and does not represent displaced intimal calcifications.*
|
||||
|
||||
|
||||
**Degenerative**
|
||||
*Lateral radiograph shows diffuse aortic calcifications <img src='img/arrows/WS.png'/> in a patient with longstanding hypertension and a dilated ascending aorta.*
|
||||
|
||||
|
||||
**Aortic Stenosis**
|
||||
*Double oblique cine MR shows a bicuspid aortic valve <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/BS.png'/> in an older patient with an ascending aortic aneurysm.*
|
||||
|
||||
|
||||
**Aortic Dissection**
|
||||
*Axial CECT shows ascending aortic false lumen dilation <img src='img/arrows/WO.png'/> in acute dissection. Note the "bird beak" sign <img src='img/arrows/BS.png'/> and "cob web" sign <img src='img/arrows/WS.png'/>, which help identify the false lumen <img src='img/arrows/WC.png'/>. 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 <img src='img/arrows/WS.png'/>. This patient was treated with emergent surgery.*
|
||||
|
||||
|
||||
**Mycotic Aneurysm**
|
||||
*Coronal CECT shows pseudoaneurysm in the mid descending aorta <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/BS.png'/> that progressed to frank rupture. Note the extravasated blood <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
**Posttraumatic Pseudoaneurysm**
|
||||
*Volume-rendered image shows focal dilation <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/BS.png'/> prior to treatment with endovascular stenting. This patient suffered high-speed deceleration trauma.*
|
||||
|
||||
@@ -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 <img src='img/arrows/CS.png'/> 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) <img src='img/arrows/CC.png'/>.*
|
||||
|
||||
|
||||
*Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches <img src='img/arrows/CS.png'/> 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) <img src='img/arrows/CC.png'/>.*
|
||||
|
||||
|
||||
*Composite axial CTA in the same patient shows the larger RAA <img src='img/arrows/CC.png'/> and smaller left aortic arch <img src='img/arrows/CO.png'/>. Note the left descending thoracic aorta <img src='img/arrows/CS.png'/>, which is typically contralateral to the dominant arch.*
|
||||
|
||||
|
||||
*Coronal CTA in the same patient shows a cephalad, larger RAA <img src='img/arrows/WS.png'/> and a more caudal, smaller left aortic arch <img src='img/arrows/WC.png'/>. 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 <img src='img/arrows/BS.png'/> causing a posterior indentation of the trachea. Also note the ascending aorta (with slab artifact) <img src='img/arrows/WO.png'/> and the proximal left aortic arch <img src='img/arrows/BC.png'/>.*
|
||||
|
||||
|
||||
*Frontal radiograph in the same patient shows mild concentric narrowing <img src='img/arrows/BS.png'/> of the midtrachea with more prominent right paratracheal nodular opacity.*
|
||||
|
||||
|
||||
*Lateral radiograph in the same patient shows the posterior tracheal indentation <img src='img/arrows/BS.png'/> 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 <img src='img/arrows/WC.png'/> and smaller left aortic arch <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/BC.png'/> and smaller left <img src='img/arrows/BS.png'/> indentations of the esophagus on frontal view due to a DAA. There is posterior indentation <img src='img/arrows/BO.png'/> 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 <img src='img/arrows/BS.png'/> vs. left <img src='img/arrows/BC.png'/> tracheal indentations on the AP reformation. Note posterior indentation <img src='img/arrows/BO.png'/> related to the RAA in the lateral reformation.*
|
||||
|
||||
|
||||
*Lateral chest radiograph in a patient with a DAA shows abnormal posterior tracheal indentation <img src='img/arrows/CS.png'/>. 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 <img src='img/arrows/CC.png'/> and left aortic arch <img src='img/arrows/CO.png'/> 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 <img src='img/arrows/CS.png'/>). There is a left descending thoracic aorta <img src='img/arrows/WC.png'/>.*
|
||||
|
||||
|
||||
*Frontal chest radiograph in a patient with a DAA with an atretic left arch shows right paratracheal opacity <img src='img/arrows/CS.png'/> related to the RAA with marked tracheal indentation <img src='img/arrows/CC.png'/>. 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 <img src='img/arrows/CS.png'/> at the thoracic inlet (i.e., 4-artery sign). Note the presence of a large RAA <img src='img/arrows/CC.png'/>, 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 <img src='img/arrows/CO.png'/> with a posteriorly tethered left subclavian artery <img src='img/arrows/CC.png'/>. 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 <img src='img/arrows/CS.png'/> and the atretic left aortic arch <img src='img/arrows/CC.png'/>, 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 <img src='img/arrows/WS.png'/> and a left aortic arch <img src='img/arrows/WC.png'/>.*
|
||||
|
||||
|
||||
*Axial chest CTA in the same patient shows that the RAA and left aortic arch have joined into 1 descending thoracic aorta <img src='img/arrows/WS.png'/>. The trachea remains slightly narrowed.*
|
||||
|
||||
|
||||
*Axial chest CTA more inferiorly shows a common left descending thoracic aorta <img src='img/arrows/WS.png'/>. The trachea now resumes a normal diameter.*
|
||||
|
||||
@@ -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) <img src='img/arrows/WO.png'/>, 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 <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Graphic demonstrates normal anatomy of the great vessels and the presence of a ductus diverticulum (DD) <img src='img/arrows/WO.png'/>, 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 <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Axial (left) and sagittal oblique (right) images in a 85-year-old woman show a broad-based DD <img src='img/arrows/CO.png'/> 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 <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WC.png'/>. 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) <img src='img/arrows/CS.png'/> with compression of the left pulmonary artery <img src='img/arrows/BS.png'/> on coronal oblique image.*
|
||||
|
||||
|
||||
*Axial CECT (left) shows spontaneous contained rupture of a 6 x 4 cm DA <img src='img/arrows/CO.png'/> with surrounding hematoma. Sagittal oblique CECT (right) shows the large DA <img src='img/arrows/CC.png'/> with small calcifications <img src='img/arrows/CS.png'/> 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 <img src='img/arrows/WS.png'/> of the anterior wall of aortic isthmus.*
|
||||
|
||||
|
||||
*Sagittal black-blood MR in the same patient shows smooth outpouching <img src='img/arrows/WO.png'/> 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 <img src='img/arrows/WS.png'/> from the anterior wall of the aortic isthmus, consistent with a typical DD.*
|
||||
|
||||
|
||||
*Axial CECT shows a partially thrombosed DD aneurysm <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/BS.png'/> and exclusion of the DA <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
*Sagittal CTA shows a smooth, well-defined wide-based outpouching <img src='img/arrows/BO.png'/> 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 <img src='img/arrows/WO.png'/>. The proximity of the DD to the main pulmonary artery <img src='img/arrows/BO.png'/> 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 <img src='img/arrows/BO.png'/> is consistent with this benign diagnosis.*
|
||||
|
||||
|
||||
*Axial CECT in the same patient shows the atypical DD <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/CC.png'/> just distal to aortic isthmus. Note postoperative replacement of ascending aorta <img src='img/arrows/WS.png'/> for type A aortic dissection.*
|
||||
|
||||
|
||||
*Oblique sagittal CTA of the thoracic aorta shows type B aortic dissection <img src='img/arrows/CS.png'/> with incidental note made of the DD <img src='img/arrows/WS.png'/> arising from a false lumen.*
|
||||
|
||||
|
||||
*VRT of the thoracic aorta shows type B aortic dissection <img src='img/arrows/CS.png'/> with incidental note made of the DD <img src='img/arrows/WS.png'/> arising from a false lumen.*
|
||||
|
||||
@@ -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"
|
||||
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|
||||
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|
||||
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|
||||
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|
||||
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|
||||
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 <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/>. CT showed abdominal fat, which had herniated into the left cardiophrenic space.*
|
||||
|
||||
@@ -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 <img src='img/arrows/WC.png'/> 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 <img src='img/arrows/WC.png'/> 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 <img src='img/arrows/BC.png'/>. 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 <img src='img/arrows/WC.png'/> as well as along the subclavian and axillary arteries bilaterally <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Axial CTA in a patient with GCA shows diffuse arterial wall thickening <img src='img/arrows/CS.png'/> and stranding of the periaortic fat. Note the reactive left pleural effusion <img src='img/arrows/CC.png'/>.*
|
||||
|
||||
|
||||
*Axial double inversion recovery FS MR in the same patient at different levels shows diffuse high signal of the aortic wall <img src='img/arrows/CS.png'/> as well as head and neck vessels <img src='img/arrows/CC.png'/>. 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 <img src='img/arrows/CS.png'/>. 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 <img src='img/arrows/CS.png'/>.*
|
||||
|
||||
|
||||
*Axial CTA in a patient with unsuspected GCA who underwent reconstruction of the ascending aorta due to aneurysm is shown. Note the aneurysmal ascending <img src='img/arrows/CS.png'/> and descending aorta <img src='img/arrows/CC.png'/>.*
|
||||
|
||||
|
||||
*Sagittal CECT MIP in the same patient shows diffuse aneurysmal thoracic aorta <img src='img/arrows/CS.png'/>. Note also the aneurysmal right brachiocephalic trunk <img src='img/arrows/CO.png'/>. 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 <img src='img/arrows/WS.png'/> and stranding of the adjacent perivascular fat due to a vasculitis. Note the stenosis of the left common carotid artery <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
*Axial GRE MR following gadolinium administration shows marked circumferential mural thickening and enhancement of the descending thoracic aorta <img src='img/arrows/WO.png'/>, 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 <img src='img/arrows/WC.png'/>, consistent with active GCA.*
|
||||
|
||||
|
||||
*Coronal contrast-enhanced MRA MIP in the same patient confirms multiple stenoses <img src='img/arrows/WC.png'/> 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 <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
*Axial CTA shows irregular mural thickening of the descending thoracic aorta <img src='img/arrows/WS.png'/> and pulmonary arteries <img src='img/arrows/WO.png'/>. Mural thickening represents a common sequela of inflammatory arteritis.*
|
||||
|
||||
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Some files were not shown because too many files have changed in this diff Show More
Reference in New Issue
Block a user