The number of operations performed for cardiovascular disease has increased since recent improvements in diagnostic and the therapeutic technology have led to a remarkable increase in the life expectancy of patients with Marfan syndrome. On the other hand, operative procedures can be difficult when patients have complications of connective tissue abnormalities such as thoracic deformities, lung diseases and ophthalmic lesions. Although recent surgical outcomes have improved, those of secondary surgery are more difficult. We describe aortic root replacement to treat perivalvular leakage after aortic valve replacement in a patient with Marfan syndrome with a severe thoracic deformity.

A 28-year-old woman with no underlying health issues was injured in a motorcycle accident and taken to our hospital by ambulance when she was 26 years old. Though she was diagnosed with multiple trauma, upon arrival at the hospital neither cardiac murmurs nor cardiac abnormalities on transthoracic echocardiography were detected. She was managed conservatively, and discharged on hospital day 16. She experienced dyspnea upon mild effort, and an early diastolic murmur appeared. She was again referred to our hospital, and diagnosed with severe aortic regurgitation. We scheduled an aortic valve replacement using an bioprosthetic valve because she intended to give birth. We also considered simultaneous aortic root enlargement as her aortic annulus was small. We performed the surgery 2 years after the initial motorcycle accident. Perioperatively, we noticed that her non-coronary cusp was torn. We converted the procedure to an aortic valve repair using an autologous pericardial patch. Her aortic regurgitation disappeared after the operation, and she was discharged on postoperative day 14. We successfully preserved the aortic valve cusps and avoided the need for anticoagulant therapy.

The UBE woven 150cc WYK graft is a non-sealed graft that became available commercially in January 2005, and does not need to be preclotted before implantation. Subjects in this study comprised 50 patients with abdominal aortic aneurysms or common iliac arterial aneurysms, who received prosthetic Y grafts in our institution. Subjects were divided into 2 groups: the U group (n=26), with implantation of the UBE graft, and the I group (n=24) who received implantation of an INTERGARD^TM woven Y graft. Intraoperative bleeding, inflammatory response and duration of postoperative hospitalization were evaluated in each group. Significant differences were noted between C-reactive protein levels, frequency of recurrence of fever and duration of postoperative hospitalization. No differences were noted between intraoperative bleeding and time. The UBE woven 150cc WYK graft, compared with the INTERGARD^TM woven Y graft, required no extra time for implantation and appeared to offer advantages such reduced immunoreaction after surgery. However, follow-up for sufficient late-phase evaluation of the grafts is required.