Combined heart transplantation and total replacement of thoracic aorta in Marfan's syndrome with recurrent aortic dissection: a case report

Abstract

It is extremely rare for a patient to need simultaneous heart transplantation (HTx) and replacement of the thoracic aorta. A 23-year-old woman with Marfan's syndrome underwent Bentall's operation and replacement of the ascending aorta (AsA) due to a type A aortic dissection (AD) in August 2001. In March 2005, she began to experience dyspnea on exertion and was found to have a huge pseudoaneurysm at the aortic root, which had caused dehiscence of the aortic conduit. In July 2009, she suffered acute chest pain followed by hypotension and cold sweating. The computed tomography (CT) scan showed a recurrent dissection with a long intimal tear extending from the arch to the mid-portion of the descending thoracic aorta (DTA). Due to technical difficulties in the repair of the aortic root, she was placed on the HTx waiting list. The next day, she received a donor heart and underwent combined HTx and total replacement of the thoracic aorta. Explantation of the heart improved the exposure of the DTA. With the use of a vascular ring connector (Vasoring), the operation was successfully performed without need for a blood transfusion. The patient was still well at 2 years after the operation. Simultaneous replacement of the heart and the whole segment of the thoracic aorta is technically possible in Marfan patients who are complicated with aortic dissection.