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Congenital heart disease
Original article
Surgical options after Fontan failure
  1. Joost P van Melle1,
  2. Djoeke Wolff2,
  3. Jürgen Hörer3,
  4. Emre Belli4,
  5. Bart Meyns5,
  6. Massimo Padalino6,
  7. Harald Lindberg7,
  8. Jeffrey P Jacobs8,9,
  9. Ilkka P Mattila10,
  10. Håkan Berggren11,
  11. Rolf M F Berger2,
  12. Rene Prêtre12,
  13. Mark G Hazekamp13,14,
  14. Morten Helvind15,
  15. Matej Nosál16,
  16. Tomas Tlaskal17,
  17. Jean Rubay18,
  18. Stojan Lazarov19,
  19. Alexander Kadner20,
  20. Viktor Hraska21,
  21. José Fragata22,
  22. Marco Pozzi23,
  23. George Sarris24,25,
  24. Guido Michielon26,
  25. Duccio di Carlo27,
  26. Tjark Ebels26
  1. 1Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
  2. 2Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
  3. 3Technical University, German Heart Center Munich, Munich, Germany
  4. 4Department of Congenital Heart Disease, Centre Chirurgical Marie Lannelongue, Paris, France
  5. 5Department of Cardiac Surgery, Catholic University Leuven, Leuven, Belgium
  6. 6Pediatric and Congenital Cardiovascular Surgery Unit, Department of Cardiac Thoracic and Vascular Sciences, University of Padova, Padua, Italy
  7. 7Department of Thoracic and Cardiovascular Surgery, Rikshospitalet, Oslo University Hospital, Oslo, Norway
  8. 8Johns Hopkins All Children's Heart Institute, All Children's Hospital and Florida Hospital for Children, Saint Petersburg, Tampa, and Orlando, Florida, USA
  9. 9Johns Hopkins University, Baltimore, Maryland, USA
  10. 10Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
  11. 11Children's Heart Centre, The Queen Silvia Children's Hospital, Gothenburg, Sweden
  12. 12Clinic for Cardiovascular Surgery, University Hospital Zurich, Zurich, Switzerland
  13. 13Leiden University Medical Center, Leiden, The Netherlands
  14. 14Academic Medical Center, Amsterdam, The Netherlands
  15. 15Department of Cardio-Thoracic Surgery, University Hospital of Copenhagen, Copenhagen, Denmark
  16. 16National Institute of Cardiovascular Disease, Children's Heart Centre Slovak Republic, Bratislava, Slovakia
  17. 17Department of Pediatric Cardiac Surgery, Children's Heart Center, Motol University Hospital, Prague, Czech Republic
  18. 18Division of Cardiac Surgery, Cliniques Universitaires Saint-Luc, Brussels, Belgium
  19. 19National Heart Hospital Sofia, Sofia, Bulgaria
  20. 20Department of Cardiovascular Surgery, Center for Congenital Heart Surgery, University Hospital Bern, Bern, Switzerland
  21. 21German Pediatric Heart Centre, Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany
  22. 22Department of Cardiothoracic Surgery, Hospital de Santa Marta, Lisbon, Portugal
  23. 23Department of Congenital and Paediatric Cardiac Surgery and Cardiology, Riuniti Hospital, Ancona, Italy
  24. 24Athens Heart Surgery Institute, Athens, Greece
  25. 25Department of Pediatric, Congenital Heart Surgery at IASO Children's Hospital, Athens, Greece
  26. 26Department of cardiothoracic surgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
  27. 27Dipartimento Medico-Chirurgico di Cardiologia Pediatrica, Ospedale Pediatrico Bambino Gesù, Roma, Italia
  1. Correspondence to Dr Joost P van Melle, University Medical Center Groningen, University of Groningen, P.O.B. 30.001, Groningen 9700 RB, The Netherlands; j.p.van.melle@umcg.nl

Abstract

Objective The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX.

Methods A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%).

Results The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (p<0.001). Fontan takedown was mostly performed in the early postoperative phase after Fontan completion, while Fontan conversion and HTX were mainly treatment options for late failure. Early (30 days) mortality was high for Fontan takedown (ie, 26%). Median follow-up was 5.9 years (range 0–23.7 years). The combined end point mortality/HTX was reached in 44.7% of the Fontan takedown patients, in 26.3% of the Fontan conversion patients and in 34.0% of the HTX patients, respectively (log rank p=0.08). Survival analysis showed no difference between Fontan conversion and HTX (p=0.13), but their ventricular function differed significantly. In patients who underwent Fontan conversion or HTX ventricular systolic dysfunction appeared to be the strongest predictor of mortality or (re-)HTX. Patients with valveless atriopulmonary connection (APC) take more advantage of Fontan conversion than patients with a valve-containing APC (p=0.04).

Conclusions Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

https://doi.org/10.1136/heartjnl-2015-309235

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