Key PointsQuestionHave the survival and risk of mortality for congenital cardiac surgery in children with tetralogy of Fallot (TOF) in Sweden changed during the past 50 years?FindingsThis cohort study including 1848 patients with TOF noted increased survival, from 68.5% to 96.0%, in children born in the 1970s compared with those born in the 2010s. The risk of mortality associated with congenital cardiac surgery decreased substantially but was 11 times higher than the risk in controls.MeaningThis study found that, despite marked success in increasing survival over the past 50 years, the overall mortality risk for patients with TOF who undergo congenital cardiac surgery appears to remain higher than in individuals without TOF.