Marfan syndrome

The Sheba Hospital in Israel operates the Center for treatment of patients with Marfan syndrome, which is the only of this kind in Israel.

Marfan syndrome is an inherited disorder that affects connective tissue.

The prevalence of this disease is 1:3-5 thousand births. Approximately 25% of patients do not have a family history of manifestations of this syndrome, is a question of a new mutation. The disease affects as a rule, cardiovascular system, eyes and musculoskeletal system.

The most common disorders of the cardiovascular system with a clear danger to the patients with Marfan syndrome are mitral valve insufficiency and aneurysm of the ascending aorta, which threatens to rupture (dissection). Aneurysm or dissection of the descending aorta, on other hand, occurs in rare cases.

Indications for surgery

The prophylactic surgery of the aorta is recommended for the patients with Marfan''s syndrome with the expansion of the aorta up to 5 cm. It is known that the risk of aortic rupture in patients with Marfan syndrome with an extended diameter of aorta up to 6 cm, which is 4 times higher than in patients with aneurysms, who do not have Marfan syndrome.

Moreover, patients whose rate of expansion of the aorta is more than 3 mm per year, as well as patients with a family history of aortic rupture, are clearly advised to undergo preventive surgery at earlier stages of enlargement of the aorta.

It is well known that dissection of the aorta is the main cause of death and disability in patients with Marfan syndrome, so the preventive operation is extremely important.