Outcome of staged surgical approach to neonates with single left ventricle and moderate size bulboventricular foramen.

Neonates with double-inlet left ventricle or tricuspid atresia with transposed great arteries and a bulboventricular foramen (BVF) area <2 cm(2)/m(2) develop BVF obstruction. This study examined the outcome of neonates with BVF area between 1 and 2 cm(2)/m(2) whose BVF was bypassed after the neonatal period. We reviewed 29 neonates with double-inlet left ventricles (n = 18) or tricuspid atresia (n = 11) and transposed great arteries. The study group consisted of 9 patients with neonatal BVF areas of 1 to 2 cm(2)/m(2) who did not undergo repair of the BVF obstruction as a neonate. The comparison group consisted of 8 "ideal" patients without BVF obstruction. Precavopulmonary shunt data from cardiac catheterization and echocardiogram and outcomes of the cavopulmonary shunt were compared. Study group patients developed a mild BVF gradient (18 +/- 10 mm Hg by cardiac catheterization) by a mean of 7 months. Left ventricular wall thickness, however, remained in the normal range (4.2 +/- 0.3 mm) and was not statistically different from the comparison group (4.1 +/- 0.4 mm). No difference was found in the precavopulmonary mean pulmonary artery pressure (15 +/- 5 vs 15 +/- 6 mm Hg), transpulmonary gradient (8 +/- 4 vs 8 +/- 5 mm Hg), and left ventricular end-diastolic pressure (7 +/- 2 vs 8 +/- 3 mm Hg). One patient in the study group died from respiratory syncytial virus pneumonia while awaiting cavopulmonary shunt. Neither group had mortality from the cavopulmonary shunt. The lengths of hospital stay were comparable (8.3 +/- 3.7 vs 8.9 +/- 6.0 days). Thus, neonates with BVF area between 1 and 2 cm(2)/m(2) develop mild but hemodynamically insignificant BVF gradient by 7 months of age. This group of patients can be managed safely with relief of BVF obstruction later in infancy.