Reconstructive surgery for congenitally malformed mitral valve.

The study aims were to evaluate early and mid-term survival and freedom from reoperation in patients with isolated mitral valve (MV) congenital malformation (MVCM) or in association with other cardiac defects, and to identify predictors for poor early and late outcome. Between January 1990 and February 2001, 58 children with MVCM underwent MV-conserving surgery; patients were allocated to group I (n = 21) with isolated MVCM, and group II (n = 37) with MVCM and associated congenital heart defects (ACHD). MV stenosis was identified in 10 (48%) group I patients, and 11 (30%) group II patients (p = NS); MV insufficiency was present in 11 (52%) patients in group I, and in 26 (70%) of group II (p = NS). The most frequently found MVCM were annular dilatation (n = 13) cases and elongated chordae (n = 14). Hammock MV was found in nine patients (15%). Six (10%) hospital deaths occurred. Mortality was 5% (n = 1) in group I, and 13% (n = 5) in group II (p = 0.4). Mean repair techniques per patient was 2.05+/-0.4 and 2.3+/-0.3 in groups I and II respectively (p = 0.009). Mechanical ventilation time was 2.1+/-1 and 2.8+/-0.7 days in groups I and II (p = 0.003). The incidence of postoperative mitral regurgitation grade > or =1 was significantly higher in group II (p = 0.008). At five-year follow up, actuarial survival was 91.5% in group I and 86% in group II (p = 0.037). Actuarial reoperation-free survival was 85% in group I and 73% in group II (p = 0.01). Multivariate analysis showed age >12 months (p = 0.033), hammock MV (p = 0.0088) and ACHD (p = 0.0048) were strong predictors for poor event-free survival. MV repair for MVCM provides acceptable early and mid-term outcome in terms of mortality and freedom from reoperation. ACHD significantly reduce early and late postoperative survival and freedom from reoperation.