Double valve replacement is associated with reduced long-term survival. This study investigates aortic valve replacement with mitral valve repair as an alternative to double valve replacement in patients with rheumatic heart disease (RHD).Between January 1995 and December 2005, 369 patients with RHD underwent combined aortic and mitral valve procedures. In 76 patients (20.6%), mitral valve repair with aortic valve replacement (group 1) was done. The remaining 293 patients (79.4%) underwent double valve replacement (group 2). A total of 351 patients (95%)--73 (96%) in group 1 and 278 (94.8%) in group 2--were 50 years of age or younger.There was no difference in early mortality in the groups (4 in group 1 versus 25 in group 2, p = 0.35). The median follow-up was 60 months (range, 6 to 132 months) and 96% complete in group 1 and 92% in group 2. Actuarial survival was 90.5% +/- 3.4% in group 1 and 81.60% +/- 2.4% in group 2 at 60 months (p = 0.07). Event-free survival at 60 months was 78.3% +/- 5.1% in group 1 and 48.4 % +/- 3.2% in group 2 (p < 0.001). Reoperation-free survival was 92.5% +/- 0.4% in group 1 and 99.5% +/- 0.05% in group 2 (p = 0.014).Mitral valve repair with aortic valve replacement provides significantly better event-free survival than double valve replacement without a better actuarial survival. Reoperation rates are higher in the mitral valve repair and aortic valve replacement group, whereas thromboembolic complications were more in the double valve replacement group. Better event-free survival in patients undergoing mitral valve repair and aortic valve replacement still argues in favor of repair of the mitral valve whenever possible.