[Surgical treatment of cardiovascular manifestations of Marfan syndrome]

Thirteen patients with cardiovascular manifestations of Marfan's syndrome (MS) were admitted to the Department of Cardiothoracic Surgery, Rigshospitalet, Copenhagen, during an 11-year period (1981-92). One patient died preoperatively. Fifteen operations were performed in 12 patients. Four operations were elective, 11 were acute. Dilatation of the aorta (> 5 cm) accompanied by insufficiency of the aortic valve was the most frequent cardiovascular pathology, (13/13 (100%)). Aortic dissection was present in 8/13 (62%) patients. At the primary operation seven patients had the ascending aorta and the aortic valve replaced by a composite graft. Two of these also had the mitral valve replaced. Four patients had the aortic valve replaced and three patients underwent CABG (coronary artery bypass grafting) due to dissection into the coronary arteries. Three patients underwent a second operation. Two of these had composite grafts implanted, the last underwent CABG. The operative mortality at the primary operation was 17%, at re-operation it was 33%. This report illustrates the high risk of acute complications in patients with MS and emphasizes the importance of an early diagnosis of the syndrome, subsequent examination of the cardiovascular system and prophylactic elective surgery. Control of blood pressure and monitoring of aortic dilatation and valve function is recommended.