Redefining Expectations of Long-Term Survival After the Fontan Procedure Twenty-Five Years of Follow-Up From the Entire Population of Australia and New Zealand

Background—The life expectancy of patients undergoing a Fontan procedure is unknown. Methods and Results—Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975–1995), 271 lateral tunnels (1988–2006), and 532 extracardiac conduits (1997–2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%–93%) for AP and 97% (95% confidence interval [CI], 94%–99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%–82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P<0.001; 95% CI, 2.4–16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New...