Leaking thoraco-abdominal aortic aneurysm in Marfan syndrome

Abstract

Marfan syndrome is a congenital disorder of the connective tissue involving the ocular, skeletal and cardiovascular systems. Cardiovascular complications account mainly for the reduced life expectancy of patients with Marfan syndrome. Thoracic aortic aneurysms usually occur as a result of proximal aortic dissection but aneurysms of the descending aorta are infrequent. We report on a patient with rupture of an extensive thoraco-abdominal aortic aneurysm (Crawford type III) with a concomitant chronic thoracic aortic dissection (Stanford type A), treated successfully with aneurysmectomy and visceral artery reconstruction. Subsequent surgical intervention for the concomitant chronic thoracic aortic dissection was also performed. The importance of life-long surveillance of the cardiovascular system is highlighted.