The first experience of one-stage total correction of the complex congenital heart disease (CHD), pulmonary atresia (PA) with ventricular septum defect (VSD) and major aortopulmonary collateral artery (MAPCA) was represented.
Total correction (TC) was performed under conditions of artificial circulation and consisted of MAPCA ligation, closure patch of VSD, reconstruction hypoplasty of PA central branches by the autologous pericardial patch and create continuity between right ventricle and neobifurcation PA using of fresh autologous pericardial valved conduit (АPVC) to prepare ex tempore. Effective method of PA-VSD-MAPCA surgical repair is one stage total correction.