Aortic intramural hematoma
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Aortic intramural hematoma is an atypical form of aortic dissection due to a contained hemorrhage into the aortic wall usually from the vasa vasorum without an intimal tear. It forms part of the acute aortic syndrome spectrum along with penetrating atherosclerotic ulcer and classical aortic dissection.
Typically, aortic intramural hematomas are seen in elderly hypertensive patients. The same condition may also develop as a result of blunt chest trauma with aortic wall injury or a penetrating atherosclerotic ulcer 1,2.
The clinical features of intramural hematoma are those of the acute aortic syndromes, namely chest pain radiating to the back and hypertension 14.
This condition is thought to begin with spontaneous rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall 2. Other theories describing the pathogenesis include thrombosis of a dissection lumen, microscopic intimal tears, progression from a penetrating atherosclerotic ulcer and traumatic medial injury 13.
The hematoma propagates along the medial layer of the aorta. Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to a communicating aortic dissection 2.
Acute intramural hematoma may coexist with other forms of acute aortic syndrome (e.g. aortic dissection) and/or hemopericardium 14.
Patients with aortic intramural hematoma and penetrating atherosclerotic ulcers have an increased risk of concomitant abdominal aortic aneurysm 13.
There is a greater predilection to involve the descending aorta 13.
Similar to aortic dissections, aortic intramural hematomas are classified according to the Stanford classification 4:
type A: involves the ascending aorta, with or without descending aortic involvement
type B: confined to the descending aorta, distal to the origin of the left subclavian artery
The DeBakey classification can also be used 5.
Acute intramural hematomas appear as focal, crescentic, high-attenuating (60-70 HU) regions of eccentrically thickened aortic wall on non-contrast CT (high-attenuation crescent sign). The narrow window width is essential for identifying subtle lesions 6. Intimal calcification may be displaced inwards, best appreciated in the non-contrast phase. Thickening is greater than the normal aortic wall thickness.
The lesions exhibit low attenuation in relation to the aortic lumen on post-contrast CT and can be far more subtle, hence a non-contrast phase before CTA is often done in an acute aortic syndrome protocol. Unlike aortic dissection, no intimal flap is present on the CTA.
On follow-up imaging, contrast can occasionally be seen within the intramural hematoma. It is important to distinguish between two entities that have different prognostic significance 11.
small region of contrast accumulation within the hematoma with invisible or small (< 2 mm) communication with the true lumen, often with a peripheral connection with an intercostal or lumbar artery 11
often regress with no adverse prognostic implications 11
ulcer-like projection: new intimal disruption (not present in the original study) with a wide neck - these usually progressively enlarge and herald a poor prognosis 11
The direction of atheromatous calcification displacement can help differentiate intramural hematoma from mural thrombus:
calcification displaced towards the aortic lumen in intramural hematomas
calcification displaced away from the aortic lumen in mural thrombus
A CTA radiology report of an intramural hematoma should include 13:
proximal and distal extent of the hematoma
the thickness of the hematoma
maximal diameter of the affected aortic lumen
presence and thickness of associated penetrating atherosclerotic ulcer
An intramural hematoma may be readily visualized with transesophageal echocardiography, which offers superior visualization of the aorta than is usually available via transthoracic examinations. Defining features include 10:
crescentic thickening of the aortic wall
normal aortic wall thickness < 3 mm
wall thickness must exceed 7 mm to diagnose intramural hematoma
wall demonstrates mixed echogenicity
predominantly echodense with scattered internal echolucencies
no internal flow detectable
lack of an intimal (dissection) flap
the luminal surface in intramural hematoma tends to be smooth and continuous
MRI may also detect the abnormality but conventional angiography will not.
Treatment and prognosis
If an intramural hematoma involves the ascending aorta (Stanford A), surgical treatment is offered to prevent rupture and progression to classic aortic dissection, which occurs in ~30% (range 20-45%) of patients 14. Medical management of type A intramural hematoma leads to mortality of 40% 13.
Conservative management is indicated for an intramural hematoma of the descending aorta (Stanford B).
77% of intramural hematomas regress at 3 years
survival of >90% at 5 years 7
Risk factors for progression of intramural hematoma and worse prognosis include 13:
presence of ulcer-like projections (ULPs)
IMH thickness greater than 10 mm
associated aortic aneurysm
increase the thickness of the intramural hematoma at follow-up CTA
Untreated, an intramural hematoma can be life-threatening as it can lead to:
The main differential diagnoses are:
thrombosed false lumen in classic aortic dissection: typically spirals longitudinally around the aorta whereas an intramural hematoma usually maintains a constant circumferential relationship with the aortic wall
aortitis: typically shows concentric uniform thickening of the aortic wall with or without peri-aortic inflammatory stranding, whereas an intramural hematoma is often eccentric in configuration
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