where do patient’s with Marfan Syndrome dissect?

24 Jan

The major cardiovascular manifestation in Marfan Syndrome is a progressive dilatation of the ascending aorta, leading to aortic aneurysm formation and eventually to fatal aortic rupture or dissection. Aortic dissection in early adult life is the leading cause of death.

The ascending and descending aorta are both abnormal in Marfan Syndrome.

· The descending aorta is affected in two out of three patients during aortic dissection, and is the site of most complications which occur during follow-up.

· Aortic dissection limited to the descending aorta can occur in patients without dilatation of the ascending aorta.

–Dissection of the descending aorta was associated with dissection of ascending aorta in 43% and was isolated in 20% of cases.

–dissections in Marfan involve descending aorta ~2/3 of the time, but these frequently involve the ascending aorta also
–if you have a patient with Marfan, and are worried about a dissection…worry about both (ascending/descending)
Submitted by J. Gullo.

Reference(s): PMID: 20232788, medscape article, picture

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