[A two staged extending graft replacement for dissecting aortic aneurysm with Marfan's syndrome]

We experienced four cases of dissecting aortic aneurysms with Marfan's syndrome, in which two staged operations were performed with satisfactory results. The operations performed in the four patients were the replacement of the ascending aorta, transverse aortic arch and the entire descending thoracic aorta in DeBakey type I dissecting aortic aneurysm, replacement of the entire descending thoracic and abdominal aorta in type IIIb, replacement of the aortic valve, ascending aorta, transverse aortic arch, the entire descending thoracic and upper abdominal aorta in type I, and replacement of the total aorta including the aortic valve in type II + IIIb, respectively. There were no operative deaths, but a 42-year-old woman with DeBakey type IIIb died suddenly 2 years 11 months after the second operation. The cause of death was presumed to be due to rupture of a dissecting aneurysm (DeBakey type II). Dissecting aortic aneurysm with Marfan's syndrome must be observed carefully and corrected surgically, because the lesion is progressive and the residual dissecting aneurysm usually dilates eventually. In view of our clinical results, we conclude that the operation for dissecting aortic aneurysm with Marfan's syndrome should be performed as extensively as possible.