<scp>T</scp>he <scp>A</scp>ustralia and <scp>N</scp>ew <scp>Z</scp>ealand <scp>F</scp>ontan <scp>R</scp>egistry: description and initial results from the first population‐based <scp>F</scp>ontan registry

<jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>The <jats:styled-content style="fixed-case">F</jats:styled-content>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.</jats:p></jats:sec><jats:sec><jats:title>Aims</jats:title><jats:p>The population is growing steadily, prompting creation of this registry to study their epidemiology, demographic trends, treatment and outcomes.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>This multicentre, binational, prospective and retrospective, web‐based registry involving all congenital cardiac centres in the region has identified nearly all <jats:styled-content style="fixed-case">F</jats:styled-content>ontan patients in <jats:styled-content style="fixed-case">A</jats:styled-content>ustralia and <jats:styled-content style="fixed-case">N</jats:styled-content>ew <jats:styled-content style="fixed-case">Z</jats:styled-content>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.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Baseline data were obtained in 1072 patients as at 1 <jats:styled-content style="fixed-case">J</jats:styled-content>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 <jats:styled-content style="fixed-case">F</jats:styled-content>ontan population is increasing steadily. Among 973 living patients, 541 (56%) gave consent for prospective collection of follow up. Between 1 <jats:styled-content style="fixed-case">J</jats:styled-content>anuary 2011 and 1 <jats:styled-content style="fixed-case">J</jats:styled-content>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.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>The population surviving after the <jats:styled-content style="fixed-case">F</jats:styled-content>ontan procedure has been growing in recent decades, especially since survival with hypoplastic left heart syndrome has improved. The <jats:styled-content style="fixed-case">A</jats:styled-content>ustralia and <jats:styled-content style="fixed-case">N</jats:styled-content>ew <jats:styled-content style="fixed-case">Z</jats:styled-content>ealand <jats:styled-content style="fixed-case">F</jats:styled-content>ontan <jats:styled-content style="fixed-case">R</jats:styled-content>egistry provides population‐based data, and only large databases like this will give opportunities for understanding the population and performing prospective trials.</jats:p></jats:sec>