title: Long-term outcomes after first-onset arrhythmia in Fontan physiology.
abstract: Patients living with a Fontan circulation are prone to develop arrhythmias. However, their prognostic impact has been seldom studied. As such, we aimed to determine the incidence and predictors of arrhythmias after the Fontan procedure and the long-term outcomes after the first onset of arrhythmias.
  
  
  
  Of the 1034 patients who have undergone a Fontan procedure as recorded in the Australian and New Zealand Fontan Registry, we identified those in whom a tachyarrhythmia or bradyarrhythmia developed. We evaluated the incidence and predictors of developing arrhythmias and their prognostic impact on late outcomes.
  
  
  
  Arrhythmia developed in 195 patients. Tachyarrhythmia was present in 162 patients, bradyarrhythmia was present in 74 patients, and both forms were present in 41 patients. At 20 years, freedom from any arrhythmia, tachyarrhythmia, and bradyarrhythmia was 66% (95% confidence interval [CI], 59-72), 69% (95% CI, 62-75), and 85% (95% CI, 80-90), respectively. On multivariable analyses, patients with an extracardiac Fontan (hazard ratio [HR], 0.23; 95% CI, 0.10-0.51; P < .001) were less likely to develop an arrhythmia, whereas those with left atrial (HR, 3.18; 95% CI, 1.45-6.95; P = .004) and right atrial (HR, 4.00; 95% CI, 2.41-6.61; P < .001) isomerism were more likely to have an arrhythmia. After onset of any arrhythmia (tachyarrhythmia or bradyarrhythmia), 10- and 15-year survivals were 74% (65%-83%) and 70% (60%-80%), respectively, and freedom from Fontan failure was 55% (44%-64%) and 44% (32%-56%), respectively. The development of any arrhythmia (HR, 2.20; 95% CI, 1-44-3.34; P < .001), tachyarrhythmia (HR, 2.56; 95% CI, 1.60-4.11; P < .001), and bradyarrhythmia (HR, 1.85; 95% CI, 1.16-2.95; P = .01) were all independent predictors of late Fontan failure on multivariable analyses.
  
  
  
  The development of an arrhythmia is associated with a heightened risk of subsequent failure of the Fontan circulation.
authors.affiliation: Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia.
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Carins
authors.affiliation: Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; The Murdoch Childrens Research Institute, Melbourne, Australia.
authors.forename: William Y
authors.initials: WY
authors.lastname: Shi
authors.affiliation: Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; The Murdoch Childrens Research Institute, Melbourne, Australia.
authors.forename: Ajay J
authors.initials: AJ
authors.lastname: Iyengar
authors.affiliation: Department of Cardiology, The Prince Charles Hospital, Brisbane, Australia.
authors.forename: Ashley
authors.initials: A
authors.lastname: Nisbet
authors.affiliation: Department of Medicine, The University of Notre Dame, Sydney, Australia.
authors.forename: Victoria
authors.initials: V
authors.lastname: Forsdick
authors.affiliation: The Murdoch Childrens Research Institute, Melbourne, Australia.
authors.forename: Diana
authors.initials: D
authors.lastname: Zannino
authors.affiliation: Green Lane Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand.
authors.forename: Thomas
authors.initials: T
authors.lastname: Gentles
authors.affiliation: Department of Cardiology, The Prince Charles Hospital, Brisbane, Australia.
authors.forename: Dorothy J
authors.initials: DJ
authors.lastname: Radford
authors.affiliation: Queensland Paediatric Cardiac Service, Lady Cilento Hospital, Brisbane, Queensland, Australia.
authors.forename: Robert
authors.initials: R
authors.lastname: Justo
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Celermajer
authors.affiliation: Paediatric Cardiology, Princess Margaret Hospital for Children, Perth, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bullock
authors.affiliation: The Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia.
authors.forename: David
authors.initials: D
authors.lastname: Winlaw
authors.affiliation: Department of Cardiology, Women's and Children's Hospital, Adelaide, Australia.
authors.forename: Gavin
authors.initials: G
authors.lastname: Wheaton
authors.affiliation: Department of Cardiology, Royal Melbourne Hospital, Melbourne, Australia.
authors.forename: Leeanne
authors.initials: L
authors.lastname: Grigg
authors.affiliation: Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; The Murdoch Childrens Research Institute, Melbourne, Australia. Electronic address: yves.dudekem@rch.org.au.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D001145:Arrhythmias, Cardiac / Q000453:epidemiology*; D001315:Australia / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D006801:Humans; D015994:Incidence; D008297:Male; D009520:New Zealand / Q000453:epidemiology; D011183:Postoperative Complications / Q000453:epidemiology*; D011379:Prognosis; D012042:Registries; D012307:Risk Factors; D016896:Treatment Outcome
keywords: Fontan failure; Fontan procedure; arrhythmia; long-term outcomes