Pulmonary atresia with ventricular septal defect (PAVSD) with major pulmonary confluens

The surgical management of pulmonary atresia with ventricular septal defect (VSD), extreme hypoplasia of the pulmonary arteries (PA), major aorto–pulmonary collaterals (MAPCAS) represents a major challenge. Two main basic concepts have been used in its management. The first is to rely mainly on the MAPCAS, unifocalizing successively the MAPCAS on one side, then the other, including the hypoplastic pulmonary arteries in the reconstruction, and then in a third stage to perform a total correction with closure of the VSD and establishment of a conduit between the right ventricle (RV) and the two unifocalization confluences [1–5]. An alternative of this classical approach in several stages, used in general in older children, has been recently proposed, consisting in a total unifocalization and complete correction in one procedure, done even early in life