[Surgical treatment of tetralogy of Fallot with pulmonary atresia: early and late results].

From 1968 to 1987, 45 consecutive patients with tetralogy of Fallot and pulmonary atresia underwent corrective surgery. In the former 10 years, there were 8 operative deaths (40%) in the 20 patients and no late death. In the recent 10 years, there were 4 operative deaths and 4 late deaths in the 25 patients. In the 17 patients associated with large aorto pulmonary collateral artery (LAPCA) which was ligated in the most patients, there were 5 operative deaths (29%) and 3 late deaths. Nine patients (53%) survived long-term. There were 7 operative deaths (25%), one late death and 20 patients (71%) being long-term survivors in the 28 patients without LAPCA. Pulmonary hemodynamics were investigated in 10 patients without LAPCA and 11 with LAPCA after corrective surgery. Pulmonary arterial mean pressure (PAm) ranged from 9-24 (17 +/- 6) mmHg in patients without LAPCA and 15-92 (37 +/- 23) mmHg in those with LAPCA. Pulmonary arteriography showed arborization abnormality in 7 of the 11 patients with LAPCA. PAm inversely correlated well with number of pulmonary artery segments connected to the central pulmonary arteries. Postoperative pulmonary hypertension was found in patients with less than 16 bronchopulmonary segments connected to the central pulmonary arteries, and two of them died of pulmonary hypertension late after surgery. These results suggested that unifocalization to connect the central pulmonary artery more than 15 bronchopulmonary segments may be an important strategy for correction of this anomaly with LAPCA.