RUPTURE OF A DISSECTING ANEURYSM INTO THE SUPERIOR VENA-CAVA IN MARFANS-SYNDROME

Aneurysmal dilatation of the aorta with subsequent dissection or ruptu re occurs frequently in patients with Marfan's syndrome. These complic ations 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 a ortic dissection and rupture into the superior vena cava occurred 8 ye ars after aortic valve replacement. The preoperative diagnosis was mad e by right heart catheterization and computed tomography. A markedly i ncreased left-to-right shunt occurred with rapid enlargement of the fi stula due to the fragility of the aortic wall characteristic of Marfan 's syndrome. Postmortem examination demonstrated severe medial necrosi s with rupture of the aortic wall into the superior vena cava which wa s adherent to the suture line of the aortotomy from the previous surge ry. Type A aortic dissection with severe congestive heart failure stro ngly suggested rupture into the pulmonary circulation.