Improved results with selective management in pulmonary atresia with intact ventricular septum.

OBJECTIVE: Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle-dependent coronary circulation and growth of the right ventricle.
METHODS: A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998.
RESULTS: Sixteen (34%) patients had a right ventricle-dependent coronary circulation, with a tricuspid valve Z-score of -3.0 +/- 0.66 versus -2.0 +/- 0.95 (P =.002) for those without a right ventricle-dependent coronary circulation. A systemic-pulmonary artery shunt only was performed in all patients with a right ventricle-dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle-dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle-dependent coronary circulation, 6 patients underwent only a systemic-pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1. 5-ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years.
CONCLUSIONS: If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair.