Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy. The size of the tricuspid valve and right ventricular cavity as well as the presence of right ventricle–dependent coronary circulation help to dichotomize between biventricular repair versus univentricular palliation or heart transplantation, as well as predicting the expected survival. The delineation and understanding of these features help to dictate both medical and surgical management.