[Subtotal aortic replacement in a patient with Marfan's syndrome--replacement of the ascending aorta and entire aortic arch for acute type I aortic dissection 4 years after the thoracoabdominal replacement].

We report a patient with Marfan's syndrome who underwent replacement of the ascending aorta and the entire aortic arch for acute type I aortic dissection 4 years after the thoracoabdominal replacement. As a result, she underwent subtotal aortic replacement from the ascending aorta to the bilateral common iliac arteries in three stages. A 48-years-old female was admitted to our service because of acute heart failure and cardiogenic shock caused by acute DeBakey type I aortic dissection. She had undergone replacement of the descending thoracic aorta at the age of 44 and replacement of the remaining thoracoabdominal aorta with reimplantation of the four major abdominal branches at the age of 46 for a chronic expanding DeBakey type IIIb dissecting aneurysm. She successfully underwent replacement of the ascending aorta and the entire aortic arch with an aortic prosthesis provided with four branch grafts for reconstruction of the arch vessels. Considering the high incidence of new aortic lesion, extensive aortic replacement and careful postoperative follow-up are necessary in patients with Marfan's syndrome.