Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries

Objective: We sought to determine the results of surgical treatment of patients with tetralogy of Fallot and Pulmonary atresia with or without major aortopulmonary collateral arteries, to clarify variables affecting early and late mortality, and to expose late, nonfatal events affecting surgical patients. Methods: The records of 495 patients operated on from 1977 to 1999 were reviewed. Patients were separated into those who did not undergo complete repair (group A) and those who did (group B). Results: Group A consisted of 160 patients. Eighty-one (51%) had palliative procedures, 45 (28%) had preliminary surgical stages (unifocalization and right ventricular outflow tract reconstruction) as initial operations, and 34 (21%) had all surgical stages but were rejected for complete repair. Early and late mortality were 16.3% (n = 26) and 23.1% (n = 31), respectively. Mean follow-up was 72.3 months. The presence of major aortopulmonary collateral arteries was a risk factor for late mortality (P = .0182). Group B consisted of 335 patients. Mean age at complete repair was 11.3 years (SD, 9.2). One hundred three (30%) patients had single-stage complete repair, whereas 232 (69%) had staged recoil struction. Twenty-two (6.6%) patients underwent reopening of the ventricular septal defect for high right ventricular pressure. Early and late mortality were 4.5% (n = 15). Risk factors were a peak right ventricular/left ventricular pressure ratio of greater than 0.7 and reopening of the ventricular septal defect (P less than or equal to .05). Late mortality was 16% (n 51). Mean follow-up was 11.4 years (SD, 7.5). Risk factors included male sex, nonconfluent central pulmonary arteries, reopening of the ventricular septal defect, and postrepair conduit exchange (n =- 137). Ten- and 20-year results were an actuarial survival of 86% and 75% and freedom from reoperation of 55% and 29%, respectively. Conclusions: Surgical repair of patients with simple or complex forms of tetralogy of Fallot with pulmonary atresia can be achieved with low early mortality. Late mortality and need for reoperation, especially conduit replacement, continue to affect the long-term well-being of these patients.