Journal article

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

Circulation | LIPPINCOTT WILLIAMS & WILKINS | Published : 2014


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..

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Awarded by National Heart and Medical Research Council (NHMRC)

Funding Acknowledgements

Dr d'Udekem is a Career Development Fellow of The National Heart Foundation of Australia (CR 10M 5339). The Australia and New Zealand Fontan Registry is funded by grants from the National Heart and Medical Research Council (NHMRC; Project Grants 1012241, 1047923, 1065794), HeartKids, and the Australian and New Zealand ( ANZ) Bank Trustees. Dr Iyengar has received postgraduate awards from the Royal Australasian College of Surgeons (Catherine Marie Enright Kelly and Eric Bishop Scholarships) and the NHMRC and The National Heart Foundation of Australia (1038802). The authors acknowledge support provided to the Murdoch Childrens Research Institute by the Victorian Government's Operational Infrastructure Support Program.