[Additional palliative operation and staged definitive operation for pulmonary atresia with intact ventricular...

Between 1981 and 1992, 38 consecutive patients underwent initial operation for pulmonary atresia with intact ventricular septum. Transpulmonary valvotomy using balloon occlusion of RV outflow was carried out in 24 patients at a mean age of 20 days. An additional Blalock-Taussig shunt was required in 4 patients early in the series. Based on a retrospective review on the patients early in the series, initial palliative procedure had been decided in conformity with the value of an index of RV development (RVDI) = RVEDV% x TVD% x RVODmm/BSAm2 x 10(-5), and recent 3 patients with RVDI < 0.35 and patent RV outflow underwent concomitant valvotomy and Blalock-Taussig shunt. Fourteen patients with muscular atresia of RV outflow or major sinusoidal coronary artery communication underwent Blalock-Taussig shunt. There were 1 operative and 3 late deaths and second Blalock-Taussig shunt was required in 6 patients. Definitive repair was carried out in 20 patients between 1985 and 1994. Fifteen patients with RVEDV > 30% of normal had biventricular repair (partial closure of ASD in 6) and 5 underwent Fontan type repair. In terms of the definitive procedures, when the RV.TV index (= RVEDV% x TVD% x 10(-4)) is greater than 0.4, complete repair should be performed. When the index is between 0.2 and 0.4, the atrial septal defect should only be partially closed when the outflow tract is reconstructed. In those cases with an index of less than 0.2, the Fontan procedure seems to be a procedure of choice.