[A case report of total aortic reconstruction and choice of staged operation in Marfan syndrome].

A 37-year-old man with Marfan syndrome underwent four operations for extensive cardiovascular disease. He was diagnosed as having AAE, AR and DeBakey type I aortic dissection. First, Bentall operation using Piehler procedure and total aortic arch replacement using retrograde cerebral perfusion and profound hypothermia at 18 degrees C were performed on May 11, 1994. Second, repair of leakage of the right coronary artery anastomosis and grafting for the descending thoracic aortic aneurysm were performed on December 3, 1994. Y-type grafting for the AAA was performed on December 21, 1996. Last, grafting for TAAA was performed under hypothermia at a rectal temperature of 20 degrees C on November 17, 1997. This surgical strategy of staged operation for extensive cardiovascular disease in Marfan syndrome is an effective method. Regular follow-up by CT is necessary for deciding the time and method of reoperation.