Rupture of a dissecting aneurysm into the superior vena cava in Marfan's syndrome

Aneurysmal dilatation of the aorta with subsequent dissection or rupture occurs frequently in patients with Marfan's syndrome. These complications are among the major causes of death. We report the case of a 51-year-old man with annulo-aortic ectasia in Marfan's syndrome. Acute aortic dissection and rupture into the superior vena cava occurred 8 years after aortic valve replacement. The preoperative diagnosis was made by right heart catheterization and computed tomography. A markedly increased left-to-right shunt occurred with rapid enlargement of the fistula due to the fragility of the aortic wall characteristic of Marfan's syndrome. Postmortem examination demonstrated severe medial necrosis with rupture of the aortic wall into the superior vena cava which was adherent to the suture line of the aortotomy from the previous surgery. Type A aortic dissection with severe congestive heart failure strongly suggested rupture into the pulmonary circulation.