The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry

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. and d'Udekem, Y. (2014) The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry. Internal Medicine Journal, 44 2: 148-155. doi:10.1111/imj.12318


Author 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.
Title The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry
Journal name Internal Medicine Journal   Check publisher's open access policy
ISSN 1444-0903
1445-5994
Publication date 2014-02
Year available 2014
Sub-type Article (original research)
DOI 10.1111/imj.12318
Open Access Status
Volume 44
Issue 2
Start page 148
End page 155
Total pages 8
Place of publication Richmond, VIC Australia
Publisher Wiley-Blackwell Publishing Asia
Collection year 2015
Language eng
Formatted abstract
 Background: The 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.

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 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.

Results: Baseline 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.

Conclusion: The 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
Keyword Exercise Capacity
Children
Adults
Multicenter
Palliation
Morbidity
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Non HERDC
School of Medicine Publications
 
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