Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.

OBJECTIVES: We sought to analyze risk factors for long-term survival (up to 36 years) after surgical repair of tetralogy of Fallot (TOF).

BACKGROUND: Survival after repair is excellent, but data >20 years are rare.

METHODS: From 1958 to 1977, 658 patients underwent correction of TOF at our institution and were analyzed for survival. Of this patient group (age 12.2 +/- 8.6 years [mean +/- SD], range 2 to 67), 39.7% had a previous palliation. Operative (n = 139) and 1-year (n = 29) deaths were excluded for long-term calculations, resulting in a study group of 490 patients.

RESULTS: Actuarial 10-, 20-, 30- and 36-year survival rates were 97%, 94%, 89% and 85%, respectively. Mortality increased 25 years postoperatively from 0.24%/year to 0.94%/year (p = 0.003). The most common cause of death was sudden death (n = 13), followed by congestive heart failure (n = 6). Multivariate correlates of impaired long-term survival were date of operation (before 1970, p = 0.0104), preoperative polycythemia (p = 0.0487) and use of a right ventricular (RV) outflow patch (p = 0.0079). Postoperative systolic RV/left ventricular pressure ratio and age showed no influence. Patients without preoperative polycythemia and an RV outflow patch (n = 164) had a 36-year actuarial survival rate of 96% and normal life expectancy.

CONCLUSIONS: Cyanosis, operative experience of the surgeon and an RV outflow tract patch influence long-term outcome after repair of TOF in older children. Early repair by experienced surgeons to avoid polycythemia and excessive RV hypertrophy is supported by this study. However, mortality risk increases 25 years postoperatively, and thus heart monitoring should be intensified.