[Surgical treatment of pulmonary atresia (PA) and critical pulmonary stenosis (PS) with intact ventricular septum (IVS)].

Patients with PA: IVS and critical PS: IVS tend to have hypoplastic right ventricles and tricuspid valves. There is a large range of hypoplasia, but the relationship between the size of the right ventricle cavity and the size of tricuspid valve ring were found to be statistically significant. Pulmonary valvotomy alone is usually not adequate for the first stage operation and a systemic-pulmonary shunt procedure often become necessary. Use of PGE1 improved the overall clinical prognosis of these patients. The appropriate operative method for the second stage operation should be determined by measuring right ventricle volume and evaluating the degree of hypoplasia of the right ventricle, tricuspid valve and pulmonary artery. In three patients with right ventricle and tricuspid hypoplasia, we performed the Glenn procedure in addition to right ventricle outflow reconstruction and obtained good surgical results.