Redefining Expectations of Long-Term Survival After the Fontan Procedure

Abstract

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 York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%–76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P <0.001; 95% CI, 2.0–7.1). Ten-year freedom from failure was 79% (95% CI, 61%–89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%–95%) for other morphologies. Conclusions— The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure.

Forward Flow Through the Pulmonary Valve After Bidirectional Cavopulmonary Shunt Benefits Patients at Fontan Operation

“…In its turn, a better Nakata index might have translated into shorter duration of the pleural drainage and shorter hospital stay after the Fontan operation in patients with APF. Furthermore, our recent study [16] demonstrated that patients staying longer in the hospital due to prolonged effusions after Fontan operation are those who would have had worse late outcomes. For those who were hospitalized for more than 18 days, the hazard of late adverse outcomes was greater.…”

Maternal and fetal outcomes of pregnancy with Fontan circulation: A multicentric observational study

“…The wide spectrum of complications includes arrhythmia, ventricular dysfunction, thromboembolic events, hepatic dysfunction, and protein losing enteropathy. Nevertheless, long-term survival is good [4,5] (90% at 10 years, 83% at 20 years, and 70% to 76% at 25 years) and an increasing number of women reaching childbearing age following surgery express desire to have children. Hemodynamic changes in pregnancy are pronounced for the abnormal heart (cardiac output and stroke volume double, myocardial oxygen consumption increases by 20%, heart rate increases by 15-20%).…”

“…However, no study has shown that anticoagulation reduces the risk in this context. Moreover, the late consequences of pregnancy on the Fontan heart need to be analyzed given the limited data on long-term survival [4,5]. Published literature includes anecdotal case reports and small series [9][10][11], which are insufficient to evaluate and identify predictors of maternal risk during pregnancy, to provide recommendations, and subsequently guide providers for counseling.…”

The Fontan Procedure for Single-Ventricle Physiology

“…However, both lateral tunnel and extracardiac Fontan procedures have better outcomes than those with atriopulmonary connections. 22 The survival rate for patients who have the Fontan operation today is 85% at 30 years. 1 Interestingly, only between 4% and 7% of patients receive a heart transplant within 20 years of the Fontan procedure.…”