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Radiology · Chest and cardiac imaging: aortic root in Marfan syndrome
Sample itemA tall young man with ectopia lentis and arachnodactyly has a CT of the thorax. Which aortic abnormality is characteristic?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Dilatation of the sinuses of Valsalva with a flask-shaped root
Fibrillin defects weaken the media, producing annuloaortic ectasia with a pear-shaped root, aortic regurgitation and a risk of dissection. The root is the site monitored by echocardiography and CT.
Option by option
A. Aneurysm confined to the distal aortic arch
Distal arch aneurysms are typically atherosclerotic or traumatic and are not the Marfan hallmark.
B. Dilatation of the sinuses of Valsalva with a flask-shaped root (correct)
Correct. Root dilatation at the sinuses is the typical Marfan lesion.
C. Linear calcification of the ascending aortic wall
Linear calcification of the ascending aorta is classically described in syphilitic aortitis.
D. Narrowing of the isthmus beyond the left subclavian artery
Isthmic narrowing is coarctation, associated with bicuspid aortic valve and Turner syndrome.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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