Mitral valve repair for congenital mitral valve stenosis in the pediatric population

Mitral valve repair is the preferred approach for congenital mitral valve stenosis in children. However, repairs in this population remain challenging.

From 2001 to 2009, mitral valve repair was attempted in 20 consecutive congenital mitral valve stenosis patients. The median age and body weight at operation were 1.4 +/- 1.9 years and 7.4 +/- 4.1 kg, respectively. Multiple level left-side heart obstruction was present in 14 patients (70%). Moderate or more mitral insufficiency was present in 5 patients.

The main etiology of the stenosis was valvular in 5 patients, supravalvular mitral ring in 4, single or predominant papillary muscle in 9, and hammock mitral valve in 2. Freedom from hospital death or transplantation was 85% (17 of 20). Two patients required replacement before discharge. In the 15 successfully repaired patients, mean mitral valve inflow pressure gradient improved from 13.9 +/- 2.7 mm Hg to 5.5 +/- 1.6 mm Hg (p < 0.0001). There were no late deaths at a mean follow-up of 46 +/- 31 months (range, 6.3 to 98.5). One patient required a second repair, and no patient has required replacement. At the last follow-up, mean mitral valve inflow pressure gradient was 7.5 +/- 4.1 mm Hg, and moderate or more mitral insufficiency was detected in 2 patients.

Mitral valve repair for congenital mitral valve stenosis can be performed in this challenging population of patients with good early survival and freedom from transplantation. Conditional follow-up for successfully repaired patients demonstrates preserved mitral valve function without need for replacement.