Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

Pulmonary atresiaPulmonary atresia/ventricular septal defectVentricular septal defect (VSD) with major aortopulmonary collateral arteriesMajor aortopulmonary collateral arteries (PA/VSDVentricular septal defect (VSD)/MAPCAs) is a rare, complex, and heterogeneous congenital heart diseasesCongenital heart disease where pulmonary blood flow is supplied by multiple MAPCAs. Physiology varies greatly depending on the number, size, and presence or absence of stenosis of the MACPAs, as well as their relationship and extent of the native pulmonary artery system. Patients can be cyanotic, balanced, or have excessive pulmonary blood flow depending on the total amount of pulmonary blood flow from MAPCAs. The goal of surgical intervention is to unifocalize all important MAPCAs to recruit as many lung segments with concomitant or subsequent achievement of biventricular physiology by closing the VSDVentricular septal defect (VSD) and establishing a right ventricleVentricles-to-pulmonary artery continuity. One stage unifocalizationUnifocalization at 4–8 months of age is a preferred approach although a subgroup of patients with diminutive branch pulmonary arteries requires a staged approach with an initial central shunt (Mee shunt). Our treatment algorithm and intraoperative pulmonary flow study to determine the feasibility of VSDVentricular septal defect (VSD) closure are included.