Fourty-two patients with pulmonary atresia and interventricular septal defect were submited to a partial or total correction, between January 1975 and October 1986, with a range of 2 months to 18 years of age. Three groups were identified: Group A: 34 patients with all bronco-pulmonary segments connected to pulmonary arteries (PA); Group B: 6 patients with some of the bronco-pulmonary segments connected to PA's; Group C: 2 patients with all bronco-pulmonary segments connected to systemic pulmonary collaterals. One to three stages were utilized to perform the proper correction. In Group A 17 were treated in one stage, with 3 deaths; 9 were submitted to a first stage where the pulmonary arteries were reconstructed with an additional Blalock-Taussig (BT) shunt, with 1 death. In two of them a second stage for total correction was performed, with no death. In the remaining 8 patients the collateral circulation was ligated and a BT shunt performed, with 1 hospital death. In Group B the first stage joining the intra or extra-pulmonary collaterals was obtained in 6 cases, without death. In two of them a total correction was performed, with 1 death. In group C a three stage operation was done in 1 patient. In the first stage an intermediate arterial segment was constructed between the lobar arteries and the BT shunt. In the second stage the contra-lateral collaterals were joined. The third stage was the establishment of the continuity between the right ventricle and the reconstructed pulmonary circulation with a good result. In the other patient of this group, until now, only the first stage was done. Serial hemodynamic studies were carried out in 32 patients. These proposed techniques demonstrate the possibility of total correction with appropriate righ-to-left ventricular pressure relation.
pulmonary atresia; ventricular septal defect; pulmonary arteries; pulmonary arteries