Background and aim of the study: Although, in patients with Marfan syndrome, the most common cardiovascular abnormalities are aortic root dilatation and aortic valve regurgitation, mitral valve dysfunction is the most frequently accompanying cardiac lesion.
Methods: The clinical course and management is reported of nine patients (seven men, two women; mean age 29.2 +/- 2.3 years) with Marfan syndrome who required different mitral valve procedures and aortic valve replacement. Mitral regurgitation was caused by annulus dilatation in all patients, by leaflet prolapse in five patients, and by chordal rupture due to endocarditis in two. Six patients underwent mitral valve replacement; three of these were operated on using preservation of the mitral valve apparatus. Three patients underwent mitral annuloplasty and chordal shortening.
Results: There was no hospital mortality, nor any valve-related complications in the series. The postoperative course was uneventful after each operation. At a mean follow up of 4.5 years, all patients were in NYHA class I or II, with improved functional capacity.
Conclusion: This experience in mitral valve procedures indicated that the connective tissue defect might compromise the results of such surgery, but that mitral valve operations could be performed satisfactorily in young Marfan syndrome patients, despite complications of left ventricular dilatation.