Abstract
During a 2 1 / 2 -year period, staged procedures of transventricular closed pulmonary valvotomy followed by balloon valvuloplasty were attempted in 12 babies with pulmonary atresia and an intact ventricular septum. All babies immediately underwent valvotomy when echocardiography revealed a tripartite right ventricle with adequate inflow and outflow dimensions and without sinusoidal-coronary arterial fistulas. After valvotomy, the overall mortality rate was 25% ( 3 / 12 ), but the only surgical death ( 1 / 12 , 8% ) was due to failure to establish continuity between the right ventricular cavity and the pulmonary trunk. The other 2 babies died of neonatal complications after successful valvotomy. Angiocardiography performed 5 to 18 months after valvotomy documented substantial growth of the right ventricular inflow and outflow dimensions in the 9 survivors. Twelve balloon dilation procedures were then performed in 7 babies. All except 1 achieved a significant drop in the right ventricular to left ventricular peak systolic pressure ratio (0.96 ± 0.40 to 0.56 ± 0.28; p < 0.01). Balloon valvuloplasty was not required in 1 baby and failed in the other, who then underwent successful right ventricular outflow tract reconstruction. After these staged procedures, follow-up at 1 month to 20 months (mean follow-up, 14.8 months) revealed resting cyanosis in 3 babies, which was related to severe residual infundibular stenosis (55 mm Hg) in 1 and a subnormal tricuspid valve annulus in 2. The remaining 5 babies (including 1 who required no valvuloplasty) were active and pink (saturation > 97%) and had a mean Doppler estimated gradient of 19 mm Hg (range, 8 to 36 mm Hg) across the pulmonary valve. Our results suggest that for babies with pulmonary atresia and intact ventricular septum, if echocardiography reveals an adequate tripartite right ventricle free from sinusoidal-coronary arterial fistulas, transventricular closed pulmonary valvotomy followed by balloon valvuloplasty is a simple biventricular staged repair with an acceptable outcome.
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