The association between ventricular septal defect and pulmonary valve atresia is a rare congenital disease, which can provoke the compression of bronchioles, that make a high mortality rate possible in the first years of life. If this syndrome is tolerated, using a convenient medical therapy, it is possible to wait for a more suitable age for a complete correction, which must provide, besides V.S.D. closing, pulmonary valvular substitution by porcine aortic heterograft. The authors also report on their own experience of five cases surgically treated and studied from a pre- and postoperative hemodinamic point of view.