Pulmonary atresia with ventricular septal defect (PAVSD) is a complex cardiopathy represented by a complete obstruction between the right ventricle outflow and the pulmonary trunk associated with a ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA). The goal of the unifocalization in the PAVSD is to prepare the pulmonary tree for the complete repair by connecting the MAPCAs to the central pulmonary arteries that should be enlarged. After that we can made the VSD or other intracardiac repair. This is a retrospective study on 31 patients. We report our results discussing the PAVSD classification and the strategy of the complete repair in comparison with other reported results.