Médianécrose aortique dans la maladie de Marfan chez l'adulte jeune. A propos de 5 cas opérés, avec 3 survies

Five cases of Marfan's syndrome with cardiovascular lesions are presented. Among these five patients, two with type I aortic dissection, one with true aneurysm of the ascending aorta and one with aortic regurgitation underwent composite grafting of the ascending aorta according to the Bentall technique. The fifth patient died preoperatively, due to extensive dissection of the transverse aorta. Marfan's syndrome is transmitted in an autosomal dominant manner. Fifty percent of the patients die at a mean age of 32 years, and 95 p. 100 of the deaths are caused by cardiovascular lesions. These lesions predominantly affect the left cardiac valves (aortic or mitral regurgitation) and the ascending aorta. Except in emergencies, replacement of the ascending aorta by a composite graft must be envisaged in subjects with Marfan's syndrome presenting with aortic regurgitation and dilatation of the aortic root.