Abstract
Described are 2 sisters in their 6th decade and a son of 1 of them, each of whom died from dissecting aneurysm of the aorta. The son died within hours but both sisters survived for some 18 months, their clinical picture being identical and closely mimicking rheumatic valvular heart disease. Necropsy findings are detailed for the 3 cases. Aortic cystic medio-necrosis was present in each. A hereditary factor with autosomal dominant transmission is responsible for idiopathic aortic cystic medionecrosis as well as for the Marfan syndrome, the stigmata of which were not present in the cases described, nor in other family members.