Cardiovascular Lesions in Arachnodactyly

The cardiovascular lesions in 34 cases of Marfan's syndrome which were studied post mortem are reviewed. Recent reports show that the most common and most important lesion is cystic necrosis of the media which results in aneurysmal formations of the ascending aorta. The aneurysm may be fusiform and the aortic ring dilated, producing a marked aortic insufficiency, or there may be a dissecting aneurysm, either recent or chronic. Valvular lesions are common but probably not important. Septal defects are occasionally encountered but are not functionally significant.