title: Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure.
abstract: OBJECTIVE
  To review our experience of patients with an atrio-pulmonary Fontan circulation to determine their long-term outcomes.
  
  
  RESULTS
  A retrospective analysis of long-term follow-up data using the Australia and New Zealand Fontan Registry was performed. There were 215 patients surviving hospital discharge after an atrio-pulmonary Fontan completion. A total of 163 patients were alive at latest follow-up, with 52 deaths. Twelve patients had required heart transplantation and 95 had Fontan failure (death, transplantation, Fontan takedown, Fontan conversion, severe systemic ventricular dysfunction or NYHA≥3). Twenty-eight year freedom from death, death and transplantation and Fontan failure were 69% (95% CI 61-78%), 64% (95% CI 56-74%) and 45% (95% CI 36-55%) respectively. One hundred and thirty patients developed atrial arrhythmias. Freedom from arrhythmia at 28years post Fontan was 22.9% (95% CI: 15.1-30.8). Development of arrhythmia increased the likelihood of death (HR:2.97, 95%CI 1.50-5.81; p=0.002), death and heart transplantation (HR:3.11, 95%CI 1.64-5.87; p<0.001) and Fontan failure (HR:4.78 95%CI 2.95-7.74; p<0.001). There were 42 patients who had thromboembolic events, of which only 12 were therapeutically anti-coagulated. Two-thirds of the surviving patients (86/126) with an intact atrio-pulmonary Fontan were regularly reviewed. Patients receiving follow-up care with general cardiologists without specialised training were more likely to face Fontan failure than those managed by cardiologists with specialist training in congenital heart disease (HR: 1.94, 95% CI 1.16-3.24; p=0.02). The majority of the surviving patients (81/86) remained physically active and almost two-thirds (54/86) were currently employed.
  
  
  CONCLUSIONS
  Two-thirds of the patients who underwent a classical atrio-pulmonary Fontan are still alive 3 decades later. The majority are affected by the burden of arrhythmias but remain functionally active today. These challenging patients should only receive follow-up care from cardiologists with specialised training.
authors.affiliation: Department of Paediatrics, University of Melbourne, Melbourne, VIC, Australia; Murdoch Childrens Research Institute, Heart Research Group, Melbourne, VIC, Australia.
authors.forename: Chin Leng
authors.initials: CL
authors.lastname: Poh
authors.affiliation: Murdoch Childrens Research Institute, Clinical Epidemiology & Biostatistics, Melbourne, VIC, Australia.
authors.forename: Diana
authors.initials: D
authors.lastname: Zannino
authors.affiliation: Department of Cardiology, Royal Children's Hospital, Melbourne, VIC, Australia.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Department of Paediatric Cardiac Surgery, The Children's Hospital at Westmead, Sydney, NSW, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Winlaw
authors.affiliation: Department of Cardiology, Royal Melbourne Hospital, Melbourne, VIC, Australia.
authors.forename: Leeanne E
authors.initials: LE
authors.lastname: Grigg
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
authors.forename: Rachael
authors.initials: R
authors.lastname: Cordina
authors.affiliation: Department of Paediatric Cardiology, Starship Children's Hospital, Auckland, New Zealand.
authors.forename: Tim
authors.initials: T
authors.lastname: Hornung
authors.affiliation: Princess Margaret Hospital for Children and Royal Perth Hospital, Perth, WA, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bullock
authors.affiliation: Queensland Paediatric and Congenital Cardiac Service, Brisbane, Lady Cilento Children's Hospital, Brisbane, Qld, Australia.
authors.forename: Robert N
authors.initials: RN
authors.lastname: Justo
authors.affiliation: Department of Paediatric Cardiology, Starship Children's Hospital, Auckland, New Zealand.
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Gentles
authors.affiliation: Department of Paediatric Cardiac Surgery, The Children's Hospital at Westmead, Sydney, NSW, Australia.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Verrall
authors.affiliation: Murdoch Childrens Research Institute, Heart Research Group, Melbourne, VIC, Australia.
authors.forename: Karin
authors.initials: K
authors.lastname: du Plessis
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Celermajer
authors.affiliation: Department of Paediatrics, University of Melbourne, Melbourne, VIC, Australia; Murdoch Childrens Research Institute, Heart Research Group, Melbourne, VIC, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, VIC, Australia. Electronic address: yves.dudekem@rch.org.au.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D000293:Adolescent; D000328:Adult; D000714:Anastomosis, Surgical / Q000379:methods; D001315:Australia / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D006325:Heart Atria / Q000601:surgery*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D009520:New Zealand / Q000453:epidemiology; D011159:Population Surveillance / Q000379:methods*; D011183:Postoperative Complications / Q000453:epidemiology*; D011651:Pulmonary Artery / Q000601:surgery*; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Adult congenital heart disease; Atrial arrhythmia; Fontan conversion; Fontan procedure; Heart failure; Hypoplastic left heart syndrome; Thromboembolism