CLINICAL-RESULTS LATE AFTER REPAIR OF TET RALOGY OF FALLOT

We studied 246 consecutive patients, mean age 11.9 +/- 6.7 years, with primary (n = 155) or secondary (n = 91) complete repair of tetralogy of Fallot (TOF) between 1961 and 1972. Prospective follow-up was compl ete and ranged from 18.1 to 29.3 (mean: 20.3 +/- 4.2) years. There wer e 46 operative and 21 late deaths. Cumulative survival was 0.76 +/- 0. 03 after 1 year, 0.72 +/- 0.03 (10 years), 0.68 +/- 0.04 (20 years) an d 0.63 +/- 0.05 (25 years). After 20 years of follow-up, which was a f ollow-up time available for all patients, cumulative complication rate s were 0.17 +/- 0.03 for documented ventricular tachycardias/fibrillat ion, 0.16 +/- 0.03 for right-heart failure, 0.13 +/- 0.03 for left-hea rt failure and 0.11 +/- 0.03 for infective endocarditis. Eighteen of t he 21 late deaths were from cardiac causes: sudden (n = 9), infective endocarditis (n = 4), left-heart failure (n = 3), and right-heart fail ure (n = 2). The hazard for ventricular arrhythmias was inconstant and increasing with time from the initial operation. After 20 years of fo llow-up, the cumulative incidence of sudden death, documented ventricu lar tachycardia/fibrillation was 0.81 +/- 10.07. Younger age at surger y resulted in a significantly better long-term prognosis (p = 0.03) wi th cumulative survival rates after 20 years being 0.90 +/- 0.06 (ages 1-9 years), 0.92 +/- 0.04 (10 to 14 years), 0.83 +/- 0.09 (15 to 19 ye ars) and 0.69 +/- 0.11 for patients being operated beyond age 20. Twen ty years following TOF repair 59.2% of the late survivors were in NYHA functional class I and 36.2% in NYHA II.