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Original research
Modes of late mortality in patients with a Fontan circulation
  1. Chin Poh1,2,
  2. Tim Hornung3,
  3. David S Celermajer4,
  4. Dorothy J Radford5,
  5. Robert N Justo6,
  6. David Andrews7,
  7. Karin du Plessis1,
  8. Ajay J Iyengar8,
  9. David Winlaw9,
  10. Yves d'Udekem1,2,9
  1. 1 Murdoch Childrens Research Institute, Parkville, Victoria, Australia
  2. 2 Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia
  3. 3 Green Lane Paediatric and Congenital Cardiac, Starship Hospital, Auckland, New Zealand
  4. 4 Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  5. 5 Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Brisbane, Queensland, Australia
  6. 6 Department of Paediatric Cardiology, Queensland Children’s Hospital, Brisbane, Queensland, Australia
  7. 7 Princess Margaret Hospital, Perth, Western Australia, Australia
  8. 8 Department of Cardiac Surgery, Royal Children's Hospital Melbourne, Parkville, Victoria, Australia
  9. 9 Heart Centre for Children, The Children's Hospital at Westmead, Sydney, New South Wales, Australia
  1. Correspondence to Dr Chin Poh, Murdoch Childrens Research Institute, Parkville, VIC 3052, Australia; poh.chinleng{at}


Objectives The mechanisms of attrition of the Fontan population have been poorly characterised and it is unclear whether some of the deaths are potentially preventable. We analysed the circumstances of late death in patients with a Fontan circulation, with a special focus on identifying lesions amenable to intervention that may have contributed to the decline of their circulation.

Methods Between 1975 and 2018, a total of 105 patients from a Bi-National Registry died beyond 1 year after Fontan completion, at a median age of 18.6 (IQR 13.8–26.0) years old, 12.7 (IQR 6.0–19.3) years after Fontan completion.

Results A total of 105 patients died—63 patients (60%) with an atriopulmonary (AP) Fontan, 21 patients (20%) with a lateral tunnel (LT) and 21 patients (20%) with an extracardiac conduit (ECC). 72 patients (69%) were reviewed within 2 years preceding death, with 32% (23/72) deemed to be clinically well. Fontan circulatory failure was the most common cause of death in 42 patients (45%). Other causes of death included sudden death/arrhythmia (19%), perioperative death (12%), neurological complication (7%) and thromboembolism (7%). All patients with an LT or ECC who died from Fontan failure had at least one surgical defect that was amenable to intervention at time of death.

Conclusions Conventional clinical surveillance has been insensitive in detecting a significant proportion of patients at risk of late death. Fontan circulatory failure contributes to half of the late deaths. Patients with an LT or ECC Fontan who died with a clinical picture of circulation failure may have potentially correctable lesions.

  • Fontan physiology
  • heart failure
  • heart transplantation

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  • Contributors All authors included have made substantial contributions to the design of the work, acquisition and interpretation of the data presented in this work.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data are available upon reasonable request.