Heart

The most serious life-threatening problems associated with Marfan syndrome involve the cardiovascular system. The two leaflets of the mitral valve may billow backwards when the heart contracts, a condition called “mitral valve prolapse”. This feature may lead to heart failure or be associated with irregularities of the heart rhythm.

The aorta (the main artery carrying blood away from the heart) is generally wider and more fragile in people with Marfan syndrome. This widening is progressive and may result in leakage of the aortic valve or in the development of a split (dissection) in the wall of the aorta. When the aorta becomes widened, medications (e.g. beta blockers, to lower blood pressure) may be prescribed, thereby reducing strain on the aorta and regulating heart rhythm. This may be followed by surgical repair preferably when the aortic root widens to 5 cms diameter, and before it becomes torn.

Patients with Marfan syndrome are recognised to have a slightly increased morbidity and mortality risk associated with general anaesthesia. Preoperative assessment should include a thorough medical examination with a chest x-ray, electrocardiogram and echocardiogram. Any treatment must be carried out in conjunction with the patient’s cardiologist.

Antibiotics may be prescribed prior to dental, genito-urinary or other minor surgical procedures, to reduce the risk of infection (endocarditis) in people who experience mitral valve prolapse, aortic valve leakage, or who have had heart surgery.

Lifestyle adaptations, such as the avoidance of strenuous exercise and contact sports, are often necessary to reduce the risk of injury to eyes, skeleton, as well as the aorta.

Beta blockers have been shown to slow the dilatation of the aortic root and their use should be considered in all patients.

Regular echocardiograms are important to monitor size and function of the heart and aorta.

Source: The Marfan Trust

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