Marfan Syndrome: Surgical Management of Cardiovascular Complications

Marfan syndrome's major manifestations involve the skeletal, ocular and cardiovascular systems. Multiple studies in patients with Marfan syndrome indicated that cardiovascular involvements may produce aneurysmal dilatation of the ascending aorta, aortic valvular regurgitation, aortic dissection, mitral valve prolapse and mitral regurgitation. These lesions are responsible for over 90% of deaths often in the third through fifth decades of life in patients with Marfan syndrome. In this paper 7 patients with Marfan syndrome, who underwent cardiovascular evaluation, were treated by combined composite valve graft replacement of the aortic root (5 patients), mechanical valve prosthesis replacement of the aortic valve and mitral valve replacement with mechanical valve prosthesis (one patient each). Patient follow-up ranged from 2 months to 4 years during which one patient died due to valve thrombosis 9 months postoperatively.