Marfan syndrome

Marfan syndrome is a connective tissue disorder characterized by unusually long limbs. The disease also affects other bodily structures—including the skeleton, lungs, eyes, heart and blood vessels—in less obvious ways. It is named for Antoine Marfan, the French pediatrician who first described it in 1896.

Treatment

The heart conditions related to Marfan syndrome may not necessarily produce obvious symptoms. As a result, regular checkups by a cardiologist are needed to monitor cardiovascular health. Potential problems may be detected through echocardiography, which involves the use of ultrasound to study the heart valves and the aorta. Beta blockers have been used to control some of the complications such as aortic aneurysms. If the dilation of the aorta threatens to lead to rupture a composite aortic valve and graft may be implanted. Although aortic graft surgery is a serious undertaking it usually results in a good outcome and a satisfactory quality of life. Elective aortic valve/graft surgey is usually considered when aortic dilatation reaches 50 millimeters, but each case needs to be specifically evaluated by a qualified cardiologist. New valve-sparing surgical techniques are becoming more common. Rupture of the aorta, or aortic dissection, is the most common cause of sudden death among Marfan syndrome sufferers.

Related Topics:
Cardiologist - Echocardiography - Ultrasound - Beta blocker - Aneurysm - Aortic dissection

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The skeletal and ocular manifestations of Marfan syndrome can also be serious, although not life-threatening. These symptoms are usually treated in the typical manner for the appropriate condition.

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