title: Heart transplantation in Fontan patients across Australia and New Zealand.
abstract: Patients with Fontan physiology may eventually require heart transplantation (HT). We determined the rates and outcomes of HT in a national, population-based multicentre study.
  
  
  
  From 1990 to 2015, 1369 patients underwent the Fontan procedure as recorded in the Australia and New Zealand Fontan Registry. We identified those who underwent HT and analysed their outcomes. We compared rates of HT between two catchment areas. In area 1 (n=721), patients were referred to the national paediatric HT programme or its associated adult programme. In area 2 (n=648), patients were referred to the national paediatric HT programme or one of the other adult HT programmes.
  
  
  
  Mean follow-up time post-Fontan was 11±8 years. Freedom from Fontan failure was 74%±3.9% at 20 years. HT was performed in 34 patients. Patients living in area 1 were more likely to have HT (4.0%, 29/721 vs 0.8%, 5/648, p<0.001) with a cumulative proportion of 3.4% vs 0.7% at 10 years and 6.8% vs 1.2% at 20 years (p=0.002). Area 1 patients were more likely to undergo HT (hazard ratio 4.7, 95% CI 1.7 to 13.5, p=0.003) on multivariable regression. Post-HT survival at 1, 5 and 10 years was 91%, 78% and 71%, respectively. Compared with other patients with congenital heart disease (n=87), Fontan patients had similar in-hospital outcomes and long-term survival.
  
  
  
  Although HT after the Fontan procedure can be achieved with excellent outcomes, most patients with Fontan failure do not undergo HT. Significant regional differences in rates of HT in Fontan patients exist.
authors.affiliation: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Australia.
authors.forename: William Y
authors.initials: WY
authors.lastname: Shi
authors.affiliation: Department of Cardiothoracic Surgery, The Alfred Hospital, Monash University, Melbourne, Australia.
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Yong
authors.affiliation: Department of Cardiothoracic Surgery, The Alfred Hospital, Monash University, Melbourne, Australia.
authors.forename: David C
authors.initials: DC
authors.lastname: McGiffin
authors.affiliation: Department of Cardiology, St Vincent's Hospital, Sydney, Australia.
authors.forename: Pankaj
authors.initials: P
authors.lastname: Jain
authors.affiliation: Greenlane Cardiothoracic Unit, Auckland City Hospital, Auckland, New Zealand.
authors.forename: Peter N
authors.initials: PN
authors.lastname: Ruygrok
authors.affiliation: Department of Cardiothoracic Surgery, The Alfred Hospital, Monash University, Melbourne, Australia.
authors.forename: Silvana F
authors.initials: SF
authors.lastname: Marasco
authors.affiliation: Greenlane Cardiothoracic Unit, Auckland City Hospital, Auckland, New Zealand.
authors.forename: Kirsten
authors.initials: K
authors.lastname: Finucane
authors.affiliation: Department of Cardiology, St Vincent's Hospital, Sydney, Australia.
authors.forename: Anne
authors.initials: A
authors.lastname: Keogh
authors.affiliation: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Australia.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
authors.affiliation: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Australia.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Australia.
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
mesh_terms: D001315:Australia; D002391:Catchment Area, Health; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000401:mortality / Q000639:trends; D054625:Healthcare Disparities / Q000639:trends; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality / Q000639:trends; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D009520:New Zealand; D017711:Nonlinear Dynamics; D011183:Postoperative Complications / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016016:Proportional Hazards Models; D012017:Referral and Consultation; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome