[Pure pulmonary atresia complicated by major sinusoidal-coronary artery communication--a case report]

The current treatment of pure pulmonary atresia consists of early initiation of prostaglandins followed by a combination of surgical procedures according to the right ventricular morphology. Major right ventricular sinusoidal-coronary artery communications (SCAC) occasionally present in patients with hypoplastic right ventricle may remain as coronary artery-right ventricular fistulae after right ventricular decompression, which may in turn be the cause for poor surgical results after pulmonary valvotomy in neonates with the SCAC. Herein, a case of this anomaly is reported with a review of literature. A 2-month-old infant underwent an aorto-pulmonary artery shunt operation using Gore-Tex graft for an emergency treatment of pure pulmonary atresia with major SCAC. The catheterization and ventriculography performed 6 months later disclosed suprasystemic right ventricular pressure and more extended SCAC. Then, right ventricular outflow tract was constructed utilizing Gore-Tex patch across the pulmonary valve under cardiopulmonary bypass. The aortico-pulmonary artery shunt was left functioning. After another 6 months, cardiac catheterization and ventriculography were repeated. Right ventricular pressure decreased to the systemic level, right ventricular ejection fraction improved, and the SCAC was not visualized by ventriculography. The decreased inter-ventricular pressure difference and improved right ventricular emptying after the second operation may have played a role in the disappearance of the SCAC. For the neonates with pure pulmonary atresia and sinusoidal-coronary artery communications, we still advocate systemic-pulmonary shunt procedure with concomitant or slightly delayed pulmonary valvotomy as the initial procedure. Unless the tricuspid valve is critically small or stenotic, there may still be a chance for the growth of the right ventricle even in this type of case.