title: Surgical options after Fontan failure.
abstract: 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.
  
  
  
  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%).
  
  
  
  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).
  
  
  
  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.
authors.affiliation: Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Joost P
authors.initials: JP
authors.lastname: van Melle
authors.affiliation: Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Djoeke
authors.initials: D
authors.lastname: Wolff
authors.affiliation: Technical University, German Heart Center Munich, Munich, Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Hörer
authors.affiliation: Department of Congenital Heart Disease, Centre Chirurgical Marie Lannelongue, Paris, France.
authors.forename: Emre
authors.initials: E
authors.lastname: Belli
authors.affiliation: Department of Cardiac Surgery, Catholic University Leuven, Leuven, Belgium.
authors.forename: Bart
authors.initials: B
authors.lastname: Meyns
authors.affiliation: Pediatric and Congenital Cardiovascular Surgery Unit, Department of Cardiac Thoracic and Vascular Sciences, University of Padova, Padua, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Padalino
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Rikshospitalet, Oslo University Hospital, Oslo, Norway.
authors.forename: Harald
authors.initials: H
authors.lastname: Lindberg
authors.affiliation: Johns Hopkins All Children's Heart Institute, All Children's Hospital and Florida Hospital for Children, Saint Petersburg, Tampa, and Orlando, Florida, USA Johns Hopkins University, Baltimore, Maryland, USA.
authors.forename: Jeffrey P
authors.initials: JP
authors.lastname: Jacobs
authors.affiliation: Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland.
authors.forename: Ilkka P
authors.initials: IP
authors.lastname: Mattila
authors.affiliation: Children's Heart Centre, The Queen Silvia Children's Hospital, Gothenburg, Sweden.
authors.forename: Håkan
authors.initials: H
authors.lastname: Berggren
authors.affiliation: Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Rolf M F
authors.initials: RM
authors.lastname: Berger
authors.affiliation: Clinic for Cardiovascular Surgery, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Rene
authors.initials: R
authors.lastname: Prêtre
authors.affiliation: Leiden University Medical Center, Leiden, The Netherlands Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Mark G
authors.initials: MG
authors.lastname: Hazekamp
authors.affiliation: Department of Cardio-Thoracic Surgery, University Hospital of Copenhagen, Copenhagen, Denmark.
authors.forename: Morten
authors.initials: M
authors.lastname: Helvind
authors.affiliation: National Institute of Cardiovascular Disease, Children's Heart Centre Slovak Republic, Bratislava, Slovakia.
authors.forename: Matej
authors.initials: M
authors.lastname: Nosál
authors.affiliation: Department of Pediatric Cardiac Surgery, Children's Heart Center, Motol University Hospital, Prague, Czech Republic.
authors.forename: Tomas
authors.initials: T
authors.lastname: Tlaskal
authors.affiliation: Division of Cardiac Surgery, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
authors.forename: Jean
authors.initials: J
authors.lastname: Rubay
authors.affiliation: National Heart Hospital Sofia, Sofia, Bulgaria.
authors.forename: Stojan
authors.initials: S
authors.lastname: Lazarov
authors.affiliation: Department of Cardiovascular Surgery, Center for Congenital Heart Surgery, University Hospital Bern, Bern, Switzerland.
authors.forename: Alexander
authors.initials: A
authors.lastname: Kadner
authors.affiliation: German Pediatric Heart Centre, Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany.
authors.forename: Viktor
authors.initials: V
authors.lastname: Hraska
authors.affiliation: Department of Cardiothoracic Surgery, Hospital de Santa Marta, Lisbon, Portugal.
authors.forename: José
authors.initials: J
authors.lastname: Fragata
authors.affiliation: Department of Congenital and Paediatric Cardiac Surgery and Cardiology, Riuniti Hospital, Ancona, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Pozzi
authors.affiliation: Athens Heart Surgery Institute, Athens, Greece Department of Pediatric, Congenital Heart Surgery at IASO Children's Hospital, Athens, Greece.
authors.forename: George
authors.initials: G
authors.lastname: Sarris
authors.affiliation: Department of cardiothoracic surgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Guido
authors.initials: G
authors.lastname: Michielon
authors.affiliation: Dipartimento Medico-Chirurgico di Cardiologia Pediatrica, Ospedale Pediatrico Bambino Gesù, Roma, Italia.
authors.forename: Duccio
authors.initials: D
authors.lastname: di Carlo
authors.affiliation: Department of cardiothoracic surgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Tjark
authors.initials: T
authors.lastname: Ebels
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005060:Europe; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D017211:Treatment Failure; D055815:Young Adult