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

Yves d'Udekem; Ajay J Iyengar; John C Galati; Victoria Forsdick; Robert G Weintraub; Gavin R Wheaton; Andrew Bullock; Robert N Justo; Leeanne E Grigg; Gary F Sholler; Sarah Hope; Dorothy J Radford; Thomas L Gentles; David S Celermajer; David S Winlaw

doi:10.1161/CIRCULATIONAHA.113.007764

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

Circulation. 2014 Sep 9;130(11 Suppl 1):S32-8. doi: 10.1161/CIRCULATIONAHA.113.007764.

Authors

Affiliations

¹ From the Department of Cardiac Surgery (Y.d., A.J.I.) and Department of Cardiology (R.G.W.), Royal Children's Hospital, Melbourne, Victoria, Australia; Murdoch Childrens Research Institute, Melbourne, Victoria, Australia (Y.d., A.J.I, J.C.G.); Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia (A.J.I., R.G.W.); Department of Mathematics & Statistics, La Trobe University, Melbourne, Victoria, Australia (J.C.G.); Heart Centre for Children, The Children's Hospital at Westmead, Sydney, New South Wales, Australia (R.G.W., G.F.S., D.S.W.); Department of Cardiology, Women's and Children's Hospital, Adelaide, South Australia, Australia (G.R.W.); Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, Western Australia, Australia (A.B); Paediatric Cardiology, Queensland Paediatric Cardiac Service, Mater Children's Hospital, Brisbane, Queensland, Australia (R.N.J.); Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia (L.E.G.); Paediatric Cardiology Services, Monash Heart, Monash Health, Melbourne, Victoria, Australia (S.H.); Department of Medicine, Monash University, Melbourne, Australia (S.H.); Adult Congenital Heart Disease Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia (D.J.R.); Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand (T.L.G.); Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia (D.S.C.); Department of Medicine, The University of Sydney, New South Wales, Australia (D.S.C.); The University of Notre Dame, Sydney, New South Wales, Australia (V.F.); on behalf of the Australia and New Zealand Fontan Registry. yves.dudekem@rch.org.au.
² From the Department of Cardiac Surgery (Y.d., A.J.I.) and Department of Cardiology (R.G.W.), Royal Children's Hospital, Melbourne, Victoria, Australia; Murdoch Childrens Research Institute, Melbourne, Victoria, Australia (Y.d., A.J.I, J.C.G.); Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia (A.J.I., R.G.W.); Department of Mathematics & Statistics, La Trobe University, Melbourne, Victoria, Australia (J.C.G.); Heart Centre for Children, The Children's Hospital at Westmead, Sydney, New South Wales, Australia (R.G.W., G.F.S., D.S.W.); Department of Cardiology, Women's and Children's Hospital, Adelaide, South Australia, Australia (G.R.W.); Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, Western Australia, Australia (A.B); Paediatric Cardiology, Queensland Paediatric Cardiac Service, Mater Children's Hospital, Brisbane, Queensland, Australia (R.N.J.); Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia (L.E.G.); Paediatric Cardiology Services, Monash Heart, Monash Health, Melbourne, Victoria, Australia (S.H.); Department of Medicine, Monash University, Melbourne, Australia (S.H.); Adult Congenital Heart Disease Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia (D.J.R.); Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand (T.L.G.); Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia (D.S.C.); Department of Medicine, The University of Sydney, New South Wales, Australia (D.S.C.); The University of Notre Dame, Sydney, New South Wales, Australia (V.F.); on behalf of the Australia and New Zealand Fontan Registry.

PMID: 25200053
DOI: 10.1161/CIRCULATIONAHA.113.007764

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.

Keywords: Fontan procedure; congenital; follow-up studies; survival.

Publication types

Multicenter Study
Research Support, Non-U.S. Gov't

MeSH terms

Adult
Australia / epidemiology
Dextrocardia / surgery
Female
Follow-Up Studies
Fontan Procedure / methods
Fontan Procedure / mortality
Fontan Procedure / statistics & numerical data*
Heart Defects, Congenital / surgery*
Humans
Hypoplastic Left Heart Syndrome / surgery
Kaplan-Meier Estimate
Male
New Zealand / epidemiology
Pacemaker, Artificial
Postoperative Complications / epidemiology
Proportional Hazards Models
Registries
Survival Analysis
Tachycardia, Supraventricular / epidemiology
Thromboembolism / epidemiology
Young Adult