Congenital mitral stenosis with or without associated defects: An evolving surgical strategy.

BACKGROUND Congenital mitral stenosis (CMS) remains a surgical challenge, particularly when it is associated with other heart defects. As in other groups of heart defects, there is a trend toward early single-stage complete repair, but the optimal surgical approach remains unanswered. METHODS AND RESULTS This study was designed to analyze the evolution of surgical strategies in patients with CMS and associated defects through single-stage and staged repair. Between 1980 and 1999, 72 children were operated on for congenital heart defects, including CMS. Preoperative transmitral gradient was 12.6+/-7 mm Hg. Preoperatively, all the patients were NYHA class III to IV. Thirteen patients had an isolated CMS; in 59, it was associated with other heart defects, mainly ventricular septal defect (n=28) or multilevel left ventricular obstruction (n=41). In this group of patients, 33 had a staged approach, and 26 had a single-stage approach. Early mortality was 12.5% (9 patients). There were no deaths in the isolated CMS and single-stage repair groups. Logistic regression revealed that early mortality was influenced by association with left ventricular outflow tract obstruction (P:<0.001) and by use of a staged approach (P:<0.01). There was no late mortality in isolated CMS; there were 2 late deaths in the group of single-stage repair and 6 late deaths in the staged approach group (P:<0.01). Reoperation was required in 24 patients, mainly for residual mitral valve dysfunction or residual left ventricular outflow tract obstruction. Including the reoperations, 10 patients received a prosthetic mitral valve. At 15 years after surgery, survival was 69.6+/-7.5%, freedom from reoperation was 70.8+/-6.3%, and freedom from mitral valve replacement was 69+/-6%. CONCLUSIONS Surgery for isolated CMS gives excellent early and long-term results. In patients with associated heart defects, a single-stage operation seems superior to a staged approach. Mitral valve replacement in this category of patients should be reserved as a salvage procedure.