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Original research
Path ahead for ‘low risk’ adolescents living with a Fontan circulation
  1. David W Baker1,
  2. Mark R Dennis1,2,
  3. Diana Zannino3,
  4. Chris Schilling4,
  5. Patricia D Moreno3,
  6. Andrew Bullock5,
  7. Patrick Disney6,
  8. Dorothy J Radford7,
  9. Tim Hornung8,
  10. Leeanne Grigg9,
  11. Yves d'Udekem3,10,
  12. Julian Ayer2,11,
  13. David S Celermajer1,2,
  14. Rachael Cordina1,2
  1. 1 Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
  2. 2 The University of Sydney Sydney Medical School, Sydney, New South Wales, Australia
  3. 3 Murdoch Childrens Research Institute, Melbourne, Victoria, Australia
  4. 4 Department of Surgery, St Vincent's Hospital Melbourne Pty Ltd, Melbourne, Victoria, Australia
  5. 5 Department of Cardiology, Perth Children's Hospital, Perth, Western Australia, Australia
  6. 6 Department of Cardiology, Royal Adelaide Hospital, Adelaide, South Australia, Australia
  7. 7 Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Brisbane, Queensland, Australia
  8. 8 Green Lane Paediatric and Congenital Cardiac Service, Starship Hospital, Auckland, New Zealand
  9. 9 Department of Cardiology, Royal Melbourne Hospital, Melbourne, Victoria, Australia
  10. 10 Department of Cardiac Surgery and Department of Cardiology, Royal Childrens Hospital Melbourne, Melbourne, Victoria, Australia
  11. 11 Department of Cardiology, Children's Hospital at Westmead, Sydney, New South Wales, Australia
  1. Correspondence to Dr Rachael Cordina, Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW 2050, Australia; rachael.cordina{at}


Objective A high risk of morbidity and mortality is well documented in adults with a Fontan circulation. The difference in outcomes between those with and without significant morbidity at the time of transition to adult care has not been well characterised.

Methods We analysed clinical outcomes in patients enrolled in the Australian and New Zealand Fontan Registry ≥16 years of age. Low risk (LR) Fontan patients were defined as those without history of sustained arrhythmia, thromboembolic event, transplantation, Fontan conversion, protein-losing enteropathy, plastic bronchitis, New York Heart Association class III/IV and/or moderate/severe atrioventricular valve regurgitation or ventricular dysfunction. Increased risk (IR) patients had one or more risk factor.

Results Inclusion criteria were met in 822 patients; mean age 26±8 years, median follow-up from age 16 was 9 years, 203 had atriopulmonary connection (APC) and 619 had total cavopulmonary connection (TCPC). Survival at 30 years was higher in the LR versus IR; 94% versus 82% (p=0.005), 89% versus 77% (p=0.07) for APC and 96% versus 89% (p=0.05) for TCPC. LR patients experienced less Fontan failure (HR 0.34, 95% CI 0.23 to 0.49, p<0.001) and ventricular dysfunction (HR 0.46, 95% CI 0.29 to 0.71, p=0.001) compared with IR patients. For LR TCPC patients, modelled survival projections at 60 years were 49%–67%.

Conclusions Clinical outcomes for adolescents LR at transition to adult care are markedly superior to those who have established risk factors for Fontan failure, which is an important consideration when formulating individualised long-term risk estimates and counselling patients.

  • Fontan physiology
  • complex congenital heart disease
  • health care delivery

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  • DWB and MRD are joint first authors.

  • Contributors All authors meet criteria for authorship based on ICMJE guidelines and contributed in the following way; conception (RC) and design of the work (all authors), data collection (DWB, MRD, DZ, CS and DPM), data analysis and interpretation (DZ, CS, DWB, MRD and RC), drafting the article (DWB, MRD and RC), critical revision of the article (all authors) and final approval of the version to be published (all authors). The lead author (RC) accepts responsibility of overall content as guarantor.

  • Funding This work was supported by an NHMRC Partnership Grant (1076849). YdU is a Clinician Practitioner Fellow of the NHMRC (1082186).

  • 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 on reasonable request. Data are available from the Australian and New Zealand Fontan registry. Written application will be reviewed by the Fontan Registry Steering Committee. Please contact or visit

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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