Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades.

BACKGROUND: Adult survivors with tetralogy of Fallot constitute a growing population with congenital heart disease. We investigated an inception cohort who underwent surgical repair. We aimed to characterize late hazard or risk for death, and determine the time-related risk of late re-operation and pulmonary valve replacement (PVR).

METHODS: All children (n=1181) with tetralogy of Fallot born before 1984 who underwent surgical repair at our institution were included. Follow-up (median 20 years after repair) was obtained from 2003 to 2006 via chart review, clinic consultation and telephone interview. Outcomes were analyzed using parametric and competing risks techniques with bagging.

RESULTS: Corrective repair performed from 1960 to 1998 included transannular patch (n=370), right ventricular outflow tract patch (n=326), no patch (n=333) and right ventricular-pulmonary artery conduit (n=54). Overall, 85+/-1% survived to adulthood (age >18 years). Thirty years after repair, survival was 80+/-1%, instantaneous hazard or risk of death was 0.5+/-0.07% per year and half of survivors had undergone surgical re-operation. Surgical era of repair did not influence late risk of death. Therefore, with early surgical mortality <2% since 1985, 20-year survival has improved to 94+/-1%. If trends in late risks match those of the earliest repairs, 40-year survival will be approximately 90% for children repaired in the modern era. Pulmonary atresia variant (n=88) was associated with three-fold higher late risk of death than classic tetralogy of Fallot (n=1069). Presence of associated branch pulmonary artery stenosis or atrioventricular septal defects conferred a less optimal late prognosis. Risk of PVR was low, constant (0.8% per year) and independent of surgical era. Both pulmonary atresia and absent pulmonary valve (n=15) variants were associated with higher risk of late re-operation or PVR. Survival after re-operation or PVR (88+/-3% and 94+/-3% at 20 years, respectively) was excellent.

CONCLUSIONS: Surgical progress has not influenced late risks for death, re-operation or PVR in adults with repaired tetralogy of Fallot. Instead, reduction of early surgical mortality to <2% is responsible for excellent late survival >90% overall. The constant risk of PVR is low and independent of repair type. Baseline morphologic features are important determinants of late outcome.