Management of cardiovascular complications in Marfan syndrome

From 1965 to 1979, 59 patients with the Marfan syndrome and cardiovascular complications underwent aortic root and aortic valve surgery. The hospital mortality was 12% (6.5% over the past 5 years). At follow-up an average of 5.1 years later, 2 patients had postoperative heart block, and late complications required reoperation in 4. There was 8 late deaths (overall mortality 25%). Survival at 6 years was 77% and improvement was noted in the functional class of 32 (74%) of the 43 survivors. 3 patients with abnormal preoperative ventricular function have continued in cardiac failure; 3 have moderate mitral regurgitation and 2 have aortic regurgitation. Aneurysms of the abdominal aorta and pulmonary artery have developed in 2 patients. Initially patients underwent surgery when their condition deteriorated despite medical treatment. Echocardiographic assessment of the aortic root has improved diagnosis and management; a large, progressively dilating aorta and evidence of ventricular dilatation were indications for surgery in 11 symptom-free patients. Although patients with Marfan syndrome are prone to other cardiovascular manifestation which affect survival, elective prophylactic surgery to the ascending aorta can be done with a low mortality and expectation of improved lifespan.