Operative results and outcomes in children with Shone's anomaly

The outcome of children with multilevel left heart obstructions with mitral valve involvement (Shone's anomaly) is generally poor. This study reviews our results and outcomes in patients with staged repair for left ventricular outflow tract obstruction (LVOTO) and mitral valve reconstruction.

A total of 27 children underwent staged repair for Shone's anomaly between 1978 and 2003. The mean age at the first operation was 9.0 months (range, 2 days to 3 years). Mitral stenosis was present in all; with supravalvular mitral ring (n = 11), mitral valve abnormalities including parachute mitral valve, fused chordae, single papillary muscle (n = 10), and "typical" (Ruckman & Van Praagh) congenital mitral stenosis (n = 14). The LVOT obstruction features included subaortic stenosis (n = 16), valvar aortic stenosis (n = 7), bicuspid aortic valve (n = 24), and coarctation (n = 18). All 27 patients underwent 94 surgical procedures, including 22 mitral operations and 48 LVOT operations.

There were no operative deaths at the first operation. Mean follow-up was 4.7 +/- 4.4 years (range, 6 months to 15 years). There were three late deaths (11%). All late deaths were secondary to severe mitral valve disease. Overall 15-year actuarial survival was 89%. All surviving patients are in New York Heart Association functional class I or II.

Patients with Shone's anomaly are seen with a wide spectrum of major anatomic and hemodynamic abnormalities. A favorable outcome is possible for most. Operative mortality is adversely affected by the severity of mitral valve disease, the degree of left ventricular hypoplasia, and the need for multiple operative procedures.