RESULTS OF CARDIOVASCULAR-SURGERY IN THE MARFAN-SYNDROME - A RETROSPECTIVE STUDY OF 49 PATIENTS

To evaluate the outcome of cardiovascular surgery in the Marfan syndro me, 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 an wa s 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. Compos ite grafts were used for aortic root reconstruction. Operative complic ations occurred in 24% of the patients. The 30-day survival was 92%. T here were five (10%) late deaths. Survival after a median of 8 years p ostoperatively was 82%. The early and late results of cardiovascular s urgery in the Marfan syndrome thus are concluded to be generally favou rable. As late reoperation frequently is needed, however, close monito ring; is advocated even after successful primary surgery.