• A J Iyengar, D S Winlaw, J C Galati, T L Gentles, R G Weintraub, R N Justo, G R Wheaton, A Bullock, D S Celermajer, and Y d'Udekem.
• Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia.
• Intern Med J. 2014 Feb 1; 44 (2): 148-55.

BackgroundThe Fontan procedure is the final in a series of staged palliations for single-ventricle congenital heart disease, which encompasses rare and heterogeneous cardiac lesions. It represents an unusual and novel physiological state characterised by absence of a subpulmonary ventricle.AimsThe population is growing steadily, prompting creation of this registry to study their epidemiology, demographic trends, treatment and outcomes.MethodsThis multicentre, binational, prospective and retrospective, web-based registry involving all congenital cardiac centres in the region has identified nearly all Fontan patients in Australia and New Zealand. Patients identified retrospectively were approached for recruitment. New recipients are automatically enrolled prospectively unless they choose to opt-out. Follow-up data are collected yearly.ResultsBaseline data were obtained in 1072 patients as at 1 January 2011. Ninety-nine patients died; 64 were lost to follow up. Forty-four per cent of patients lost were between 20 and 30 years of age. The size of the Fontan population is increasing steadily. Among 973 living patients, 541 (56%) gave consent for prospective collection of follow up. Between 1 January 2011 and 1 January 2013, an additional 47 subjects were enrolled prospectively. The current proportion of patients operated with hypoplastic left heart syndrome is currently 29% and is growing rapidly.ConclusionThe population surviving after the Fontan procedure has been growing in recent decades, especially since survival with hypoplastic left heart syndrome has improved. The Australia and New Zealand Fontan Registry provides population-based data, and only large databases like this will give opportunities for understanding the population and performing prospective trials.© 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.

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