title: The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry.
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.
  
  
  OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: A J
authors.initials: AJ
authors.lastname: Iyengar
authors.forename: D S
authors.initials: DS
authors.lastname: Winlaw
authors.forename: J C
authors.initials: JC
authors.lastname: Galati
authors.forename: T L
authors.initials: TL
authors.lastname: Gentles
authors.forename: R G
authors.initials: RG
authors.lastname: Weintraub
authors.forename: R N
authors.initials: RN
authors.lastname: Justo
authors.forename: G R
authors.initials: GR
authors.lastname: Wheaton
authors.forename: A
authors.initials: A
authors.lastname: Bullock
authors.forename: D S
authors.initials: DS
authors.lastname: Celermajer
authors.forename: Y
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D000293:Adolescent; D000328:Adult; D001315:Australia / Q000453:epidemiology; D016208:Databases, Factual; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000379:methods / Q000706:statistics & numerical data; D006301:Health Services Needs and Demand; D006330:Heart Defects, Congenital / Q000145:classification / Q000453:epidemiology / Q000601:surgery; D006801:Humans; D008134:Long-Term Care / Q000379:methods / Q000706:statistics & numerical data; D008297:Male; D009520:New Zealand / Q000453:epidemiology; D017063:Outcome Assessment, Health Care; D010166:Palliative Care / Q000379:methods / Q000706:statistics & numerical data; D011184:Postoperative Period; D012042:Registries / Q000706:statistics & numerical data; D012189:Retrospective Studies
keywords: Fontan procedure; congenital; heart defect; outcome assessment (healthcare); registry; research design