The Marfan syndrome and cardiac surgery

Of 31 patients with Marfan's syndrome and cardiovascular complications, 25 had ascending aortic aneurysms, five with aortic dissection; 26 had aortic regurgitation, two with aortic stenosis; and eight had mitral regurgitation, five with aortic regurgitation. Surgery included prosthetic aortic valve replacement in 24 patients and aortic valvular bicuspidization in two; 19 had resection of aneurysm with Dacron tube replacement, three had lateral aneurysmorrhaphy, and two had circumferential strip excision with end-to-end anastomosis. Four patients underwent mitral valve replacement. Operative complications occurred in 10 patients. There were nine (29 percent) hospital deaths, but only one death occurred in 12 patients operated upon since 1970. Late complications included prosthetic leak in six patients (23 percent) with reoperation in five; all survived. Five late deaths occurred (16 percent); one was unrelated and one was of unknown cause. Although risk of cardiac surgery remains high, our recent results support an aggressive surgical approach, particularly in patients with advanced or deteriorating cardiovascular complications.