Impact of cardiovascular operation on survival in the Marfan patient.

This report is concerned with the surgical treatment of cardiovascular manifestations of Marfan syndrome in 151 patients by 280 operations, 70 before referral, 203 by the authors during a 16-year period, and seven by others in the follow-up interval. This therapy resulted in mitral valve replacement in 13, aortic valve replacement in 135, aortic segment replacement in 151 (including total aortic replacement in 10), nearly total aortic replacement in 26, entire thoracic aortic replacement in seven, and replacement of the entire descending and abdominal aorta in 20 patients. The early (30-day) survival was 94%, and the 5- and 10-year Kaplan-Meier survival was 75% and 56%, respectively. Fusiform aneurysms were present in the ascending aorta, aortic arch, or both in 135, the descending aorta in two, the thoracoabdominal aorta in four, and the abdominal aorta in seven. Aortic dissection occurred in 102 (67%) patients, and 34 (22%) patients with ascending aortic operations required reoperation. This study suggests that the results of current techniques warrant a more aggressive approach to these problems to permit better long-term results.