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Original research
Reintervention and survival in 1428 patients in the Australian and New Zealand Fontan Registry
  1. Michael Daley1,2,
  2. Karin du Plessis2,
  3. Dianna Zannino2,
  4. Tim Hornung3,
  5. Patrick Disney4,
  6. Rachael Cordina5,6,
  7. Leeanne Grigg7,
  8. Dorothy J Radford8,9,
  9. Andrew Bullock10,
  10. Yves d'Udekem1,2,11
  1. 1 Cardiac Surgery, Royal Children's Hospital Melbourne, Melbourne, Victoria, Australia
  2. 2 Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia
  3. 3 Green Lane Paediatric and Congenital Cardiac, Starship Hospital, Auckland, New Zealand
  4. 4 Department of Cardiology, Royal Adelaide Hospital, Adelaide, South Australia, Australia
  5. 5 Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  6. 6 Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia
  7. 7 Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia
  8. 8 Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia
  9. 9 Faculty of Medicine, The University of Queensland, Brisbane, Queensland, Australia
  10. 10 Children's Cardiac Centre, Princess Margaret Hospital for Childrens, Perth, Western Australia, Australia
  11. 11 Department of Paediatrics, The University of Melbourne, Melbourne, Victoria, Australia
  1. Correspondence to Dr Yves d'Udekem, Cardiac surgery, Royal Childrens Hospital Melbourne, Melbourne, VIC 3052, Australia; yves.dudekem{at}


Objective Patients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients.

Methods A retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival.

Results Of the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%.

Conclusions A substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.

  • congenital heart disease
  • complex congenital heart disease
  • fontan physiology
  • congenital heart disease surgery

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  • Contributors All authors contributed to the production of this manuscript through various methods including: study design, data collection, data analysis, manuscript preparation, revisions and preparation of final manuscript and revisions.

  • Funding This work was supported by a National Health and Medical Research Council (NHMRC) Partnership grant (1076849). The Murdoch Children’s Research Institute received support through the Victorian Government’s Operational Infrastructure Support Program. Yd'U is a NHMRC Clinician Practitioner Fellow (1082186).

  • Disclaimer Yves d’Udekem is a consultant for Merck Sharpe & Dohme and Actelion. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

  • Competing interests None declared.

  • Patient consent for publication Not required.

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

  • Data availability statement No data are available.