One hundred consecutive patients with the Marfan syndrome underwent composite graft repair of an ascending aortic aneurysm between September 1976 and June 1989. Twenty-two patients had ascending aortic dissection at the time of composite graft repair; 18 patients also had a mitral valve procedure. There were no hospital deaths among 92 patients undergoing elective repair. One of 8 patients undergoing emergency repair of a ruptured aneurysm died in the operating room. The overall hospital mortality rate was 1%. There have been ten late deaths among the 99 hospital survivors (10.1%). Five deaths occurred among the first 11 patients in this series and five occurred among the last 88 patients (5.7%). Three late deaths resulted from composite graft endocarditis; 3 other patients with endocarditis are alive after aortic root replacement with cryopreserved homografts. Late coronary dehiscence caused death in 1 patient and was successfully repaired in a second. Actuarial survival for the 100 patients was 92.6% at 5 years and 75.8% at 10 years. Currently, composite graft repair of Marfan aneurysms of the ascending aorta can be performed with low hospital and late mortality. Marfan aneurysms with a diameter of 6 cm or greater should be repaired with the Bentall composite graft procedure, even if the patient is asymptomatic.
