The Marfan syndrome: surgical technique and follow-up in 50 patients.

Fifty patients with Marfan's syndrome underwent operation at the National Heart Hospital because of cardiovascular complications. Forty-six had an aneurysm of the ascending aorta, 13 had chronic dissection, and 6 had acute dissection of the aortic wall. Forty-three aortic valves were incompetent, and five were stenotic and incompetent. One mitral valve had minor regurgitation. The Starr-Edwards prosthesis was used in 36 patients, homograft valves in 4, fascia lata valves in 2, and xenograft valves in 6. The ascending aorta was replaced with a Dacron tube in 40 patients and with an aortic homograft in 2. Three patients required Dacron patches over the aneurysm, and 1 patient had plication of the aortic wall. Early mortality totaled 12% (6 patients). Only 1 of these patients died in the last five years. Reoperations for homograft incompetence, periprosthetic leak, and acute dissection of an unreplaced aorta resulted in 1 hospital death (33%). Forty-three patients have been followed for up to 8.5 years (mean, 3.5 years), with 7 late deaths (46.2%). The improvement in recent surgical results with decreased operative mortality supports an aggressive surgical approach to Marfan's syndrome in view of the poor prognosis for the natural history of this disease.