Combined Heart Transplantation and Total Replacement of Thoracic Aorta in Marfans Syndrome With Recurrent Aortic Dissection: A Case Report

Transplantation Proceedings, 05/08/2012

A 23–year–old woman with Marfan's syndrome underwent Bentall's operation and replacement of the ascending aorta due to a type A aortic dissection 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 scan showed a recurrent dissection with a long intimal tear extending from the arch to the mid–portion of the descending thoracic aorta. The next day, she received a donor heart and underwent combined heart transplantation and total replacement of the thoracic aorta. With the use of a vascular ring connector, the operation was successfully performed without need for a blood transfusion. The patient was still well at 2 years after the operation.

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