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T he A ustralia and N ew Z ealand F ontan R egistry: description and initial results from the first population‐based F ontan registry
Author(s) -
Iyengar A. J.,
Winlaw D. S.,
Galati J. C.,
Gentles T. L.,
Weintraub R. G.,
Justo R. N.,
Wheaton G. R.,
Bullock A.,
Celermajer D. S.,
d'Udekem Y.
Publication year - 2014
Publication title -
internal medicine journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 1444-0903
DOI - 10.1111/imj.12318
Subject(s) - medicine , ventricle , population , prospective cohort study , hypoplastic left heart syndrome , heart disease , surgery , pediatrics , environmental health
Background The F ontan 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. Aims The population is growing steadily, prompting creation of this registry to study their epidemiology, demographic trends, treatment and outcomes. Methods This multicentre, binational, prospective and retrospective, web‐based registry involving all congenital cardiac centres in the region has identified nearly all F ontan patients in A ustralia and N ew Z ealand. 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. Results Baseline data were obtained in 1072 patients as at 1 J anuary 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 F ontan population is increasing steadily. Among 973 living patients, 541 (56%) gave consent for prospective collection of follow up. Between 1 J anuary 2011 and 1 J anuary 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. Conclusion The population surviving after the F ontan procedure has been growing in recent decades, especially since survival with hypoplastic left heart syndrome has improved. The A ustralia and N ew Z ealand F ontan R egistry provides population‐based data, and only large databases like this will give opportunities for understanding the population and performing prospective trials.