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title: "Aortic Aneurysm: Rupture"
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docid: "e64e6a27-0c8d-4b4f-8ca5-d2e65c9f2e9a"
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authors:
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- key: "b00d2bdb-66e1-41ed-90b4-c52904f4d598"
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value: "Seth Kligerman, MD, MS"
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- key: "9ad9af12-61a1-44d6-af52-0ee1f38eb298"
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value: "Davis Vigneault, MD, DPhil"
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- key: "5de0df07-7b3e-4678-8767-1519e1153f29"
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value: "Dominik Fleischmann, MD"
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breadcrumbs:
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-
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name: "Cardiac"
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slug: "cardiac"
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treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
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-
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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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-
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name: "Aortic Aneurysm: Rupture"
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slug: "aortic-aneurysm-rupture"
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treeNodeId: null
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category: "Cardiac"
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documentVersionId: "fa15c37f-7c00-4ecc-b261-17c3e427a8a2"
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imageCount: 14
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lastUpdated: "01/24/25"
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pageDescription: "Aortic Aneurysm: Rupture"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Aortic Aneurysm: Rupture"
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pageTitle: "Aortic Aneurysm: Rupture | STATdx"
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enhancedTitle: "Aortic Aneurysm: Rupture"
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type: "DX"
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references: true
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breadcrumbs:
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- "Cardiac"
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- "Diagnosis"
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- "Aorta"
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- "Aortic Aneurysm: Rupture"
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---
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# KEY FACTS
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- ## Terminology
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- 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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- 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
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- AAA growing ≥ 1 cm in 1 year
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- CTA findings of rupture
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- Hemomediastinum, hemopericardium, or hemothorax, hemoperitoneum depending on site
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- Focal discontinuity in aneurysm wall or disruption of otherwise circumferential aortic calcifications
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- ## Top Differential Diagnoses
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- AA without rupture
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- Acute aortic syndrome with rupture
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- Aortic pseudoaneurysm
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- Large vessel vasculitis or inflammatory aortitis (IgG4 or Erdheim-Chester disease)
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- ## Clinical Issues
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- 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
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- Ascending aortic aneurysm with rupture
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- Open repair
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- Arch aneurysm with rupture
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- Open repair with arch replacement
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- Descending thoracic or abdominal aortic aneurysm with rupture
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- Thoracic endovascular aortic repair (TEVAR) or open surgical repair
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# TERMINOLOGY
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- ## Definitions
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- 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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- ## General Features
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- ### Best diagnostic clue
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- Enlarged thoracic aorta with associated hemomediastinum, hemopericardium, or hemothorax is highly suggestive of ruptured thoracic aortic aneurysm (TAA)
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- ### Location
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- Abdominal aortic aneurysm (AAA) > TAA
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- TAA most common in ascending thoracic aorta
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- However, most ruptures involve AAA descending TAA
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- ### Size
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- Risk of rupture or dissection often linked to aneurysm size, morphology, and rate of growth
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- Thoracic aneurysms
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- Aneurysm ≥ 6 cm was significantly associated with risk of rupture or dissection
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- Yearly rate of rupture or dissection ≥ 6 cm ranges from 10-15%
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- At ≥ 7 cm, rate dramatically increases to > 40%
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- Ascending aortic aneurysms (AAs)
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- For sporadic aortic root and ascending AAs, repair now recommended in certain patients with aneurysm size ≥ 5 cm
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- 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
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- Descending thoracic aorta
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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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- 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
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- AAA
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- Risk of 5-year rupture 20-40% with aneurysms > 5 cm
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- Elective repair for asymptomatic patients with AAA diameter of ≥ 5.5 cm and ≥ 5 cm in men and women, respectively
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- For symptomatic patients, should be repaired regardless of size
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- TAA is commonly defined as maximal thoracic diameter ≥ 4.0 cm
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- Some define TAA as maximal thoracic diameter ≥ 4.5 cm and maximal thoracic diameter ≥ 4.0 and < 4.5 cm as "dilated"
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- In reality, upper limits of normal is dependent upon age, sex, body size, and aortic segment
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- To account for this, z-scores and indexed metrics have been introduced, though adoption in adult populations has been limited
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- 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
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- 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
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- Sinus of Valsalva measurements may be made from sinus-to-sinus or cusp-to-commissure
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- As sinus-to-sinus measurements tend to be larger than cusp-to-commissure measurements, method used should be specified
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- If sinuses are largely symmetric, single averaged measurement may be reported
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- If sinuses are asymmetric, all 3 measurements should be reported
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- Rate of growth should be assessed when serial imaging is available
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- ### Morphology
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- May be fusiform or saccular with former being more common and latter associated with increased risk of rupture
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- Wall calcification and mural thrombus are common and may be extensive
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- Aortic tortuosity may complicate surgical approach and should be described
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- Rate of growth
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- 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
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- Repair recommended for those with heritable thoracic aortic disease or bicuspid aortic valve and growth ≥ 0.3 cm in 1 year
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- Repair recommended if AAA is rapidly expanding ≥ 1 cm in 1 year
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- ## Radiographic Findings
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- Mediastinal widening may be seen in ruptured or unruptured TAA but is often difficult to appreciate
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- Pleural effusion (hemothorax) if ruptured into pleural space
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- Pericardial effusion (hemopericardium) if ruptured into pericardial space
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- Following thoracic endovascular aortic repair (TEVAR), endograft migration, kinking, or fracture may be identified
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- ## CT Findings
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- ### NECT
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- In patients unable to receive contrast, TAA morphology and diameter may be assessed on NECT
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- Signs of frank or impending rupture, such as hemomediastinum and intramural hematoma, are both evident on NECT
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- Following TEVAR, NECT is also useful for distinguishing calcified mural thrombus from endoleak
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- ### CTA
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- Impending, contained, or frank rupture
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- Hemomediastinum, hemopericardium, or hemothorax depending on location of rupture
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- Focal discontinuity in aneurysm wall or disruption of otherwise circumferential aortic calcifications
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- Dense crescent sign: Intramural hematoma or dissection of acute blood into mural thrombus
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- Lysis of thrombus with expansion of contrast-enhanced flow lumen
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- ## MR Findings
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- Can provide much of same information as CTA without ionizing radiation
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- Following TEVAR, MR is most useful in setting of nickel titanium (nitinol) grafts, which do not produce susceptibility artifacts
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- LGE of excluded aneurysm sac is sensitive for detecting endoleaks
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- Time-resolved MR angiography and 4D phase-contrast MR (4D flow) are useful in further characterizing endoleaks
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- ## Echocardiographic Findings
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- 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
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- 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
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- ## Imaging Recommendations
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- ### Best imaging tool
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- NECT + CTA
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- ### Protocol advice
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- CTs should be reconstructed using thin (submillimeter) slices to allow for high-quality multiplanar reconstruction (MPRs)
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- NECT is useful for identifying blood products, and, following endovascular repair, for distinguishing calcified mural thrombus from endoleak
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- ECG gating is crucial for accurate evaluation aortic root and ascending aorta
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- If ECG gating is performed retrospectively, multiphase images may be reconstructed enabling assessment of aortic valve function
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- Delayed imaging is useful for fully opacifying flow lumen in very large aneurysms and for identifying endoleaks occult on arterial phase
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- Screening
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- 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
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- Screening should image entire aorta either by CTA or MRA
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- Screening echocardiography is often indicated
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- Surveillance
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- Measurements should be performed at same location, measurement technique, and imaging modality
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- Patients with newly identified TAA or those near treatment threshold should be imaged more frequently (every 6 months)
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- Patients with stable TAA may be imaged less frequently (every 1-3 years according to individual risk)
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# DIFFERENTIAL DIAGNOSIS
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- ## Aortic Aneurysm Without Rupture
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- AAs are not uncommon, especially in older patients
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- Coexistent pericardial and pleural effusions are not uncommon in patients with TAA due to underlying cardiovascular disease
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- In some patients, TAAs with complex ulcerated plaque with coexistent simple transudative pleural and pericardial effusions could potentially mimic rupture
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- ## Acute Aortic Syndrome With Rupture
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- Aortic dissection, intramural hematoma, or penetrating atherosclerotic ulcer can all lead to aortic rupture
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- Presence of intimal injury is characteristic of AAS
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- If rupture occurs in AAS, it is due to disruption of residual media and overlying adventitia
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- In AAs with rupture, rupture is through all 3 layers of aortic wall
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- ## Aortic Pseudoaneurysm
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- Aortic dilation contained by overlying adventitia due to vascular injury
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- Commonly secondary to trauma, surgery, or infection
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- More likely to be saccular, able to expand, and change shape rapidly
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- ## Large Vessel Vasculitis or Inflammatory Aortitis (IgG4 or Erdheim-Chester Disease)
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- Patients often symptomatic with chest/back pain, weight loss, and elevated inflammatory markers
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- Areas of stenosis often with coexistent aneurysm
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- Circumferential wall thickening in areas, often associated with enhancement
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- Inflammation in periaortic fat could mimic rupture
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# PATHOLOGY
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- ## General Features
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- Cystic medial degeneration results in progressive loss of elastin in tunica media
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- Aortic wall stress increases with aortic diameter (governed by Laplace's Law)
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- Rupture may occur in TAA with disruption of all 3 layers of aortic wall
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- Rupture may also occur in setting of acute aortic syndrome
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- Intimal injury is precipitating cause
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- Rupture occurs through residual media and adventitia
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- Recognition of rupture is more important than differentiating between etiologies, although different etiologies may lead to different methods of repair
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Chest pain, hemorrhagic shock, tamponade
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- Unruptured TAAs are usually asymptomatic and identified incidentally on imaging
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- ### Other signs/symptoms
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- Dysphagia secondary to esophageal compression (dysphagia aortica)
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- Unilateral hoarseness secondary to mass effect on left recurrent laryngeal nerve (Ortner's syndrome)
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- Dyspnea secondary to airway compression
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- Back pain secondary to erosion of vertebral bodies
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- ## Demographics
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- TAA occurs in 5-10 per 100,000 person-years
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- Risk factors for TAA rupture include
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- Large diameter (especially > 6.0 cm)
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- Rapid aneurysm growth (≥ 0.5 cm/year)
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- Saccular morphology
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- Female sex
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- Hereditary or infectious etiology
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- ## Natural History & Prognosis
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- Once ruptured, usually fatal without repair
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- Rate of growth and risk of rupture are highly dependent upon underlying etiology as aforementioned
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- ## Treatment
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- Ascending AA with rupture
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- Open repair
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- Arch aneurysm with rupture
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- Open repair with arch replacement
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- Descending thoracic or AAA with rupture
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- TEVAR or open surgical repair
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- TEVAR showed lower mortality and reduced risk of paraplegia, stroke, and hypovolemic shock compared to open repair
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7f6ab856-7757-41e0-8d94-a19884f2f9db
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## References
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# Selected References
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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## Images
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### Selected Images
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*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'/>.*
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*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'/>.*
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*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'/>.*
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*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.*
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*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'/>.*
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*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.*
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*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'/>.*
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*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.*
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*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.*
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*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.*
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*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.*
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### Additional Images
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*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.*
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*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.*
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*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'/>.*
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*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.*
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Reference in New Issue
Block a user