Pulmonary Atresia with Intact Ventricular Septum in the Neonate: A Review.

Pulmonary atresia with intact ventricular septum is a cyanotic congenital heart lesion comprised of complete obstruction of the pulmonary outflow and varying degrees of right heart hypoplasia. There is a wide spectrum of disease from patients requiring minimally invasive transcatheter pulmonary valve perforation/balloon dilation as a neonate and having biventricular circulation, to patients requiring a ductal arterial stent or systemic-to-pulmonary-artery shunt as a neonate and undergoing single ventricle palliation, or even primary heart transplant. Factors determining these pathways include anatomic elements of the right ventricle, and presence, degree and severity of right ventricular-dependent coronary circulation.