Right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum: a case report.

Pulmonary atresia with intact ventricular septum (PAIVS) is a morphologically heterogeneous lesion and accounts for 1-3% of critically ill infants with congenital heart disease. Numerous surgical approaches have been attempted with varying degrees of success, but the mortality rate is still high in most series. The optimal surgical procedure depends on the size and morphology of the tricuspid valve and right ventricle and the presence or absence of right ventricle-dependent coronary circulation.