title: The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes.
abstract: OBJECTIVE
  To identify factors associated with hospital and long-term outcomes in a binational cohort of extracardiac conduit (ECC) Fontan recipients.
  
  
  METHODS
  All patients who underwent an ECC Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified, and perioperative, follow-up, echocardiographic and reintervention data collected. Risk factors for early and late mortality, failure and adverse outcomes were analysed.
  
  
  RESULTS
  A total of 570 patients were identified, and late follow-up was available in 529 patients. The mean follow-up was 6.7 years (standard deviation: 3.5) and completeness of the follow-up was 98%. There were seven hospital mortalities (1%) and 21 patients (4%) experienced early failure (death, Fontan takedown/revision or mechanical circulatory support). Prolonged length of stay occurred in 10% (57 patients), and prolonged effusions in 9% (51 patients). Overall survival at 14 years was 96% (95% confidence interval [CI]: 93-98%), and late survival for patients discharged with intact Fontan was 98% (95% CI: 94-99%). The rates of late failure (late death, transplantation, takedown, New York Heart Association class III/IV or protein-losing enteropathy) and adverse events (late failure, reoperation, percutaneous intervention, pacemaker, thromboembolic event or supraventricular tachycardia) per 100 patient-years were 0.8 and 3.8, and their 14-year freedoms were 83% (95% CI: 70-91%) and 53% (95% CI: 41-64%), respectively. After adjustment for confounders, hypoplastic left heart syndrome (HLHS) was strongly associated with prolonged effusions (OR: 2.9, 95% CI: 1.4-5.9), late failure (hazard ratio [HR]: 2.8, 95% CI: 1.1-7.5) and adverse events (HR: 3.6, 95% CI: 1.3-7.5).
  
  
  CONCLUSIONS
  The extracardiac Fontan procedure provides excellent survival into the second decade of life, but half of patients will suffer a late adverse event by 14 years. Patients with HLHS are at higher risk of late adverse events than other morphological groups, but their survival is still excellent.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, VIC, Australia Murdoch Childrens Research Institute, Melbourne, VIC, Australia Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, VIC, Australia ajayjiyengar@gmail.com.
authors.forename: Ajay J
authors.initials: AJ
authors.lastname: Iyengar
authors.affiliation: Heart Centre for Children, The Children's Hospital at Westmead, Sydney, NSW, Australia University of Sydney, Sydney, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Winlaw
authors.affiliation: Murdoch Childrens Research Institute, Melbourne, VIC, Australia Department of Mathematics and Statistics, La Trobe University, Melbourne, VIC, Australia.
authors.forename: John C
authors.initials: JC
authors.lastname: Galati
authors.affiliation: Department of Cardiology, Women's and Children's Hospital, Adelaide, SA, Australia.
authors.forename: Gavin R
authors.initials: GR
authors.lastname: Wheaton
authors.affiliation: Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand.
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Gentles
authors.affiliation: Department of Cardiology, The Royal Melbourne Hospital, Melbourne, VIC, Australia.
authors.forename: Leeanne E
authors.initials: LE
authors.lastname: Grigg
authors.affiliation: Paediatric Cardiology, Queensland Paediatric Cardiac Service, Mater Children's Hospital, Brisbane, QLD, Australia.
authors.forename: Robert N
authors.initials: RN
authors.lastname: Justo
authors.affiliation: Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, QLD, Australia.
authors.forename: Dorothy J
authors.initials: DJ
authors.lastname: Radford
authors.affiliation: Murdoch Childrens Research Institute, Melbourne, VIC, Australia Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, VIC, Australia Department of Cardiology, Royal Children's Hospital, Melbourne, VIC, Australia.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, WA, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bullock
authors.affiliation: University of Sydney, Sydney, Australia Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Celermajer
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, VIC, Australia Murdoch Childrens Research Institute, Melbourne, VIC, Australia Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, VIC, Australia.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D001315:Australia; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects / Q000379:methods*; D006330:Heart Defects, Congenital / Q000601:surgery; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000503:physiopathology*; D015994:Incidence; D008297:Male; D009520:New Zealand; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
keywords: Congenital heart disease; Disease-free survival; Follow-up studies; Fontan procedure; Retrospective studies; Survival rate