Surgical management of mitral regurgitation associated with Marfan's syndrome.

BACKGROUND: The surgical treatment of mitral regurgitation associated with Marfan's syndrome remains controversial because of the underlying degenerative process.

METHODS: From October 1986 to June 1996, 33 patients with Marfan's syndrome underwent a mitral valve procedure. The mean age was 30 years (range, 2 to 55 years). Mitral regurgitation was caused by annulus dilatation in 2 patients, leaflet prolapse in 30 patients, and restricted leaflet motion in 1 patient. Mitral valve repair was performed in all patients except 1 who had mitral valve replacement.

RESULTS: Two patients died in the perioperative period. All survivors were available for follow-up, which ranged from 1 month to 122 months (mean follow-up, 39 months). There were three late deaths, two of which were related to aortic complications. The actuarial survival rate was 78.9% at 10 years. Freedom from mitral valve reoperation was 87.1% at 10 years. Echocardiographic studies were obtained in all survivors and showed absent or mild (1+) mitral regurgitation in 21 patients and moderate (2+) mitral regurgitation in 3.

CONCLUSIONS: Mitral valve repair for mitral regurgitation in patients with Marfan's syndrome can be performed safely in almost all instances. This technique provided stable midterm results comparable with those obtained for other degenerative mitral valve diseases.