446 lines
34 KiB
Markdown
446 lines
34 KiB
Markdown
---
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title: "Aortic Intramural Hematoma"
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docid: "128bc4cc-a26d-47d5-90e7-b1a1f608e657"
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authors:
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- key: "b66f94a2-4335-4ce8-a3ba-8c5527f8774c"
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value: "Domenico Mastrodicasa, MD"
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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 Intramural Hematoma"
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slug: "aortic-intramural-hematoma"
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treeNodeId: null
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category: "Cardiac"
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documentVersionId: "df9b047f-b83f-46c7-85da-2cecf3b24727"
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imageCount: 23
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lastUpdated: "12/19/24"
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pageDescription: "Aortic Intramural Hematoma"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Aortic Intramural Hematoma"
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pageTitle: "Aortic Intramural Hematoma | STATdx"
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enhancedTitle: "Aortic Intramural Hematoma"
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type: "DX"
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references: true
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ddx: 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 Intramural Hematoma"
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---
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# KEY FACTS
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- ## Terminology
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- 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
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- IMH as aortic dissection (AD) variant such as AD with thrombosed false lumen or AD with small entry and absent reentry tear
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- 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.
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- ## Imaging
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- NECT: Crescentic or eccentric aortic wall hyperdensity
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- CECT
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- Crescentic or eccentric aortic wall thickening
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- ↓ luminal aortic diameter along (i.e., candy cane) IMH extension
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- Penetrating aortic ulcer (PAU): Outpouching of contrast beyond expected aortic margin in setting of atherosclerosis; penetrates internal elastic lamina
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- Ulcer-like projections (ULP): Outpouching of contrast with wide intimal opening (i.e., > 3 mm), limited to intima
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- PAU appears similar to ULP; PAU is often surrounded by IMH, which has smooth interface with lumen
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- Intramural blood pool (IBP)
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- Pool of contrast with small luminal communication (1-2 mm)
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- Often has communication with aortic branch (e.g., intercostal/lumbar arteries)
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- ## Top Differential Diagnoses
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- Aortitis (Takayasu and giant cell arteritis)
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- Aortic aneurysm with mural thrombus
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- ## Pathology
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- Stanford classification
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- Type A (~ 40%): Ascending ± descending aorta
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- Type B (~ 60%): Descending aorta
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- ## Clinical Issues
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- Abrupt onset of severe chest or back pain, hypertension
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- Predictors of poorer prognosis and ↑ mortality
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- Stanford type A
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- Maximum aortic diameter (ascending: 48-55 mm; descending: 40-41 mm)
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- IMH thickness 10-11 mm
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- PAU
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- Treatment
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- Type A IMH: Surgical treatment
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- Type B IMH: Medical treatment and close follow-up
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# TERMINOLOGY
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- ## Abbreviations
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- Intramural hematoma (IMH), aortic dissection (AD)
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- ## Definitions
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- 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
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- IMH as AD variant
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- AD thrombosed false lumen or AD with small entry and absent reentry tear
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- IMH as purely descriptive term referring to often localized hematoma ("bruise") in aortic wall
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- Associated with broad range of acute aortic pathologies, including PAU, LIT, traumatic aortic injuries, rupturing aneurysms, iatrogenic dissections, etc.
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- IMH (or class 2 AD)
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- Absent or very small entry tear and absent reentry tear
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- PAU (or class 4 AD)
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- Ulcerated atherosclerotic lesion that penetrates internal elastic lamina into media ± IMH
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Aortic wall hyperdensity on NECT can be focal, crescentic, elongated, &/or circumferential
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- ## Radiographic Findings
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- ### Radiography
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- Often normal chest radiograph
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- Interval radiography: Displacement of intimal calcification
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- ## CT Findings
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- ### NECT
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- Focal, crescentic, elongated, &/or circumferential aortic wall hyperattenuation
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- Nonclotted (flowing) blood → 30-45 HU; acute thrombus → 50-90 HU
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- In first 7-10 days, acute thrombus is hyperdense relative to blood because it retracts (↑ hemoglobin concentration)
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- Pitfalls: Anemia, residual contrast medium; left ventricular myocardium can be used as reference
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- Narrow window width and level settings helpful to recognize hyperattenuating IMH
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- Can be seen with LIT in ascending aorta
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- ### CTA
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- Crescentic or eccentric aortic wall thickening, smooth lumen-wall interface
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- ↓ luminal aortic diameter along IMH extension
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- better seen on long-axis reformations (e.g., candy cane views)
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- Often, no discrete intimomedial flap seen on CT but can be identified surgically or pathologically in nearly all cases
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- Identification of intimomedial flap by CT represents coexistent AD
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- Common ancillary findings: Pericardial effusion and periaortic hematoma (↑ risk of rupture)
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- Common features of IMH (dissection variant)
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- Intramural blood pool (IBP)
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- a.k.a. branch pseudoaneurysms
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- Pool of contrast with no obvious or very small luminal communication (1-2 mm)
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- Communications with aortic branches (e.g., intercostal arteries); MIP reformations helpful to show communications
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- Usually not associated with worse prognosis [unlike ulcer-like projection (ULP)]
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- ~ 50% resolve on follow-up; risk of incomplete regression: Larger size &/or visible connection to intercostal/lumbar arteries
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- ULP
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- 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
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- Contrast outpouching with wide (> 3 mm) connection to flow lumen
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- New intimal injury in area of high shear stress w/o atherosclerosis
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- Descending aorta > ascending aorta > arch (like PAU)
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- Usually not noted at initial CTA; can develop in ~ 1/3 of patients within 1-4 months
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- Implies poor prognosis and unfavorable outcome (↑ risk of evolution to dissection, rupture or, often saccular aneurysmal dilation)
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- Highest risk: ULP > 20-mm diameter, > 15-mm depth, or in ascending aorta/arch
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- Differentiation from penetrating aortic ulcer (PAU): Lack of irregular intima/atherosclerosis, no IMH, not seen initially; can be indistinguishable
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- PAU: Intramural blood can be associated with acute PAU
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- Intimal disease in severe atherosclerosis; ulcer-like plaque burrows through intima into media
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- May be associated with focal or segmental IMH
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- Results in wall thickening; smooth outer bulge related to vessel remodeling
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- PAU + IMH > 20-mm max diameter, > 10-mm max depth, aneurysm, or increasing size on follow-up: ↑ risk of progression → consider repair
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- Limited intimal tear (LIT): Intramural blood can be associated with LIT
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- Outpouching of contrast typically along ascending thoracic aorta, distal to origin of left coronary artery
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- Absence of visible intimomedial flap
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- ## MR Findings
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- ### T1WI
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- Acute: Isointense; subacute: Hyperintense
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- ### T2WI
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- Acute: Hyperintense; subacute: Lower intensity
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- ### T1WI C+
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- Lack of enhancement
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- ### MRA
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- Efficiently shows PAU, ULP, and IBP
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- Phase-contrast MR
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- Lack of flow unless coexistent AD
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- ## Echocardiographic Findings
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- ### Echocardiogram
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- Transesophageal echocardiography is equivalent to CT and MR in aortic root but limited elsewhere
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- ## Imaging Recommendations
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- ### Best imaging tool
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- CT most cost effective and fastest to perform
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- ### Protocol advice
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- Always include thin-slice NECT and CTA
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# DIFFERENTIAL DIAGNOSIS
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- ## Aortitis (Takayasu and Giant Cell Arteritis)
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- Inflammation of large and medium-sized arteries
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- Scattered areas of stenosis ± aneurysm
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- Parietal thickening simulates IMH on CTA
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- Not hyperdense on NECT
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- Mural enhancement on MR or delayed CT with contrast
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- ## IgG4 and Erdheim-Chester Disease Aortitis or Periaortitis
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- Circumferential enhancing aortic or periaortic soft tissue
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- Hyperintense on NECT
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- Focal or diffuse
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- Aneurysmal dilation or lumen stenosis less common
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- IgG4 > ECD
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- Infiltrating periaortic soft tissue
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- Also perirenal infiltration in ECD
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- ## Aortic Aneurysm With Mural Thrombus
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- Mural thrombus in lumen of dilated aorta and not in wall
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- [Aortic Dissection](/document/aortic-dissection/57e3428e-1f18-4f38-95c6-f7fe2d93c00a)
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- Clinically, IMH presentation may be identical to AD or LIT
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- AD with thrombosed false lumen may simulate IMH on CTA
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- Thrombosed false lumen is typically not hyperdense on NECT; AD is also irreversible
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- LIT with subadventitial hematoma may be indistinguishable from IMH
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- LIT often lacks ↓ luminal aortic diameter, may have periaortic fluid, and may exhibit discrete external aortic bulge (aortic stretch mark)
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- ## Aortic Sarcoma
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- Extremely rare
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- Difficult to differentiate from exophytic atherosclerotic plaque
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- Noncalcified discrete aortic wall mass
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Medial degeneration with isolated intimal tear (small, usually undetectable by CT), contained hemorrhage in vessel media, no exit tear
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- Causes: Spontaneous vasa vasorum rupture, microscopic intimal tears, PAU, thrombosed false lumen of AD, trauma
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- It is now accepted that communications with medial hematoma do exist
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- ### Associated abnormalities
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- May be associated with thoracic or abdominal aortic aneurysm
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- Development of ULP, PAU, IBP; can enlarge, progress to frank AD, rupture
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- ## Staging, Grading, & Classification
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- Stanford classification
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- Type A (~ 40%): Ascending aorta ± descending aorta
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- Type B (~ 60%): Excludes ascending aorta
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- ## Gross Pathologic & Surgical Features
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- PAU
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- Ulcerated atherosclerotic plaque
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- May progress to aortic rupture
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- IMH
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- Represent intimal defect too small on CT but nearly always seen at surgery or gross pathology
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Abrupt onset of severe chest or back pain
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- Hypertension
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- 10-30% of all acute aortic syndromes
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- Presentation can be indistinguishable from AD and LIT
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- ### Other signs/symptoms
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- Diaphoresis, hypotension, and tachycardia
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- ## Demographics
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- ### Age
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- 7th decade
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- ### Sex
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- M = F
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- ### Epidemiology
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- Prevalence of Marfan syndrome/connective tissue disorders not as high as AD
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- ## Natural History & Prognosis
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- Evolution patterns of IMH
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- Spontaneous resolution (~ 10%)
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- Evolution or coexistence with AD (28-47%)
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- Aortic rupture (20-45%)
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- Stability over time (rare)
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- Predictors of poorer prognosis and ↑ mortality
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- Dilated aorta: ascending 48-55 mm, descending 40-41 mm
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- IMH thickness > 10-11 mm
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- PAU in acute phase: ↑ incidence of progression
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- Especially when PAU > 20 mm
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- Stanford type A
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- Mortality of type A IMH with only medical treatment is ~ 40%
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- IBP: Not considered at ↑ risk for disease progression
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- Often regresses over time
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- ## Treatment
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- Similar to that for typical AD
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- Type A IMH: Surgical treatment
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- Asian factor: Good results reported with initial medical management for type A IMH in Asian countries
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- Type B IMH: Medical treatment and close follow-up
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- Endovascular or open surgery reserved for complications
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- PAU should be treated early (e.g., endovascular or surgery)
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- IBP usually observed and follow-up CTA
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1dc2c8f0-8031-44c2-a1b9-8665ab78da55
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## References
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# Selected References
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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## Differential diagnosis
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### Aortic Intramural Abnormality
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DDX:75d7b37f-bc37-493b-8961-8b2a9001fb94
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## Images
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### Selected Images
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*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.*
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*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.*
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*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.*
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*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.*
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*The orange overlay represents the hyperattenuating IMH. The light green overlay marks the thickened, atherosclerotic intima.*
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*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.*
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*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.*
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*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.*
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*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.*
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*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.*
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*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.*
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*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'/>.*
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*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.*
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*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'/>.*
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*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.*
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*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'/>.*
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*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.*
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### Additional Images
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*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.*
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*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.*
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*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.*
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*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.*
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*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.*
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*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'/>.*
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*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.*
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