Results of cardiovascular surgery in the Marfan syndrome. A retrospective study of 49 patients.

Abstract

To evaluate the outcome of cardiovascular surgery in the Marfan syndrome, the records of 49 patients (median age 35 years) who underwent 60 operations were reviewed. Primary surgery was elective in 39 patients and emergency in ten. Non-dissecting aneurysm with diameter 4-19 cm was present in 34 cases and distal, isolated aneurysm in four. In eight cases there was type A acute aortic dissection with median diameter 5.0 cm. One patient was operated on for mitral valve insufficiency, one for ventricular septal defect and one (acute) for endocarditis. Composite grafts were used for aortic root reconstruction. Operative complications occurred in 24% of the patients. The 30-day survival was 92%. There were five (10%) late deaths. Survival after a median of 8 years postoperatively was 82%. The early and late results of cardiovascular surgery in the Marfan syndrome thus are concluded to be generally favourable. As late reoperation frequently is needed, however, close monitoring is advocated even after successful primary surgery.