Abstract 16372: Redefining Expectations of Long-Term Survival After the Fontan Procedure: 25 Years of Follow-Up From the Entire Population of Australia and New Zealand
Background: Late life expectancy of patients undergoing a Fontan procedure is unknown.
Methods and Results: The follow-up of all 991 survivors of the 1091 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from each participating centre. The population consisted of 237 atrio-pulmonary connections (AP; 1975-1995); 291 lateral tunnels (LT; 1988-2006) and 563 extra-cardiac conduits (ECC; 1997-2011). The proportion of patients with hypoplastic left heart syndrome increased from 5/340 (2%) before 1990 to 63/289 (22%) after 2000.
Survival 25 years after AP Fontan was 73% (95% CI 62 - 82%). Fifteen-year survival was 86% (95% CI 79 - 90%) for AP, 93% for LT (95% CI 89 - 96%) and 97% for ECC (95% CI 94 - 99). By multivariable analysis, AP Fontan independently predicted worse survival when compared to ECC (HR=4.7, p=0.015, 95% CI 1.3-16.2).
Freedom from failure at 15 and 20 years was 82% (95%CI 77 - 85%) and 70% (95%CI 62 - 76%): the Fontan circulation was considered to have failed if patients died (n=51), required heart transplantation (n=17), were reoperated for take-down (n=7) or for conversion from AP to ECC (n=28), were in NYHA class III or IV (n=11) or were diagnosed with protein-losing enteropathy (n=16). By multivariable analysis, hypoplastic left heart syndrome was the primary predictor of Fontan failure: HR=4.8; p=0.011; 95% CI 1.4-14.3. Ten-year freedom from failure was 81% (95% CI 66 - 90%) for patients with hypoplastic left heart syndrome vs 91% (95% CI 89 - 93%) for patients with other morphologies.
Conclusion: The long-term survival of the Australia and New Zealand Fontan population is better than expected. Patients with an atrio-pulmonary connection experience survival of 73% 25 years after Fontan. With current surgical techniques, long-term survival in this population-based registry exceeds 95%. Patients with hypoplastic left heart syndrome are likely to achieve worse survival because they are more prone to fail.
- © 2013 by American Heart Association, Inc.