Thoracic aortic aneurysm in Marfan's syndrome.

Thoracic aortic aneurysms are the main cardiovascular complication of Marfan's syndrome. Elastic tissue dystrophy of the ascending aorta is responsible for appearances of cystic medial necrosis in the major forms. Dilatation of the ascending aorta is progressive with time. The risk of dissection and rupture, an acute complication of Marfan's syndrome, is very high when the aortic dilatation reaches 60 mm, although dissection may be observed with dilatation of 50 mm. Aortic regurgitation is found in half the cases. Echocardiography, CT scan and MRI provide accurate assessment of the anatomy of the lesions and help in following up the dilatation. Betablocker therapy slows down the progression of the dilatation. Bentall's procedure was first described in 1968 with its technical variants for reimplantation of the coronary arteries : Cabrol's procedure, technique of periostal lean-to, the button technique, are major advances in the surgical treatment of ascending aortic aneurysms. The life expectancy is reported to be about 90 % at 5 years and 75 to 80 % at 10 years. Reoperation is sometimes necessary because of pseudo-aneurysms occurring as a late complication at the operation site or because of other aneurysms developing. Progress in the field of genetics is a hope for the future.