title: Management of cardiovascular complications in Marfan syndrome.
abstract: 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.
mesh_terms: D000293:Adolescent; D000328:Adult; D001021:Aortic Valve / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000601:surgery; D002318:Cardiovascular Diseases / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000401:mortality / Q000503:physiopathology; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000601:surgery; D013514:Surgical Procedures, Operative / Q000401:mortality