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

Abstract

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

Methods: 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.

Results: 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.

Conclusions: 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.