THE BENTALL PROCEDURE IN PATIENTS WITH MARFANS-SYNDROME

From 1985 to 1996, 18 patients with Marfan's syndrome underwent the Be ntall procedure at Mount Sinai Hospital. They are compared with 38 pat ients aged < 40 without Marfan's syndrome who also underwent composite valve-graft replacement of the ascending aorta. The mean age of the n on-Marfan group was 33 while that of the Marfan group was 29. Nearly t hree-quarters of each group were male. Aortic dissection was present i n 33% of the Marfan group but only in 18% of the other group. Seventy- eight percent of the Marfan patients and 89% of the non-Marfan patient s had aortic regurgitation. Twenty-one percent of the patients in the control group nevertheless had gross aortic pathology suggestive of Ma rfan's syndrome and may have had variants of the syndrome; 26% of the non-Marfan group had a bicuspid aortic valve. A modification of the Be ntall procedure with implantation of coronary artery buttons was perfo rmed in the majority of the patients in both groups. Three patients, a ll in the group with Marfan's syndrome, required a concomitant mitral valve procedure. There was one death in each group. Two non-Marfan pat ients required reoperation; neither had dissection. Four patients with Marfan's syndrome underwent reoperation for distal disease in the aor ta; they comprised one third of the Marfan patients who had aortic dis section. Three late complications occurred in the group with Marfan's syndrome: progressive cardiomyopathy; myocardial infarction; and late tamponade. There were also two late sudden deaths in the group with Ma rfan's syndrome, which may have been the consequence of aortic rupture . No difference in immediate operative mortality following the Bentall procedure was noted between patients with and without Marfan's syndro me, but young patients without Marfan's syndrome seem to have better e vent-free and long-term survival. In patients with Marfan's syndrome, the presence of acute dissection makes reoperation more likely, and su dden death from rupture still occurs despite careful postoperative sur veillance. A higher incidence of severe mitral valve disease was found among young patients with Marfan syndrome than in controls.