Pulmonary atresia with intact ventricular septum and coronary cameral fistulae: an unusual finding of a subsystemic right ventricle.

Abstract We report the case of a neonate with pulmonary atresia with intact ventricular septum and coronary cameral fistulae despite having a subsystemic right ventricle. We review the literature on coronary cameral fistulae in this disease and right ventricle-dependent coronary circulation. We discuss the potential consequences of this physiology, including risk of adverse cardiovascular events that may impact risk stratification and surgical palliation.