Surgical treatment for cardiovascular lesions of patients with Marfan syndrome.

OBJECTIVE: Without treatment, the life expectancy of patients with Marfan syndrome is reduced by the associated cardiovascular abnormalities. In this study, we reviewed our experience of the surgical treatment of this condition to identify the primary indication for surgical repair and the optimal surgical management.

METHODS: Between January 1986 and December 2000, 44 patients underwent surgery for Marfan syndrome at Shizuoka City Hospital. There were 28 male and 16 female patients, with a mean age of 40.9 +/- 15.0 years. Nineteen of the patients had developed type A dissection of the aortic arch. Three patients underwent remodelling of the aortic root. Ten patients required multiple repeat surgical interventions.

RESULTS: There were four early and five late deaths. The survival rate at 1, 5, and at 10 years was 90.9%, 80.5%, and 74.3%, respectively. Freedom from postoperative events, which included cardiac death, additional surgery, and new or repeat aortic dissection at 1, 5, and at 10 years was 93.0%, 69.1%, and 53.2%, respectively. Among patients having type A dissection, the mean annual enlargement in the diameter at the level of the proximal descending aorta was 4.0 +/- 4.0 mm. Postoperative echocardiography with Doppler studies was performed in series on patients who had received remodelling of the aortic root, with subsequent mild aortic regurgitation in 2 patients and moderate regurgitation in 1 patient.

CONCLUSIONS: Repeated postoperative studies with CT scans and earlier surgical intervention before the onset of aortic dissection are mandatory for improvement in the long-term survival rate of patients with Marfan syndrome. A valve-sparing procedure for a deformed aortic valve is promising, although the long-term results are not yet clear.