title: Favourable mid-term outcome after heart transplantation for late Fontan failure.
abstract: OBJECTIVE
  Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF.
  
  
  METHODS
  Twenty-year multi-institutional retrospective analysis on OHT for FF.
  
  
  RESULTS
  Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality.
  
  
  CONCLUSIONS
  OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.
authors.affiliation: Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, Netherlands g.michielon@umcg.nl.
authors.forename: Guido
authors.initials: G
authors.lastname: Michielon
authors.affiliation: Department of Cardiology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands.
authors.forename: Joost P
authors.initials: JP
authors.lastname: van Melle
authors.affiliation: Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, Netherlands.
authors.forename: Djoeke
authors.initials: D
authors.lastname: Wolff
authors.affiliation: DMCCP, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
authors.forename: Duccio
authors.initials: D
authors.lastname: Di Carlo
authors.affiliation: Congenital Heart Institute of Florida, St Petersburg, FL, 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 Center, The Queen Silvia Children's Hospital, Goteborg, Sweden.
authors.forename: Hakan
authors.initials: H
authors.lastname: Berggren
authors.affiliation: Rikshospitalet, University Hospital Oslo, Oslo, Norway.
authors.forename: Harald
authors.initials: H
authors.lastname: Lindberg
authors.affiliation: Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy.
authors.forename: Massimo A
authors.initials: MA
authors.lastname: Padalino
authors.affiliation: Department of Cardiac Surgery, University Hospital UZ Leuven, Leuven, Belgium.
authors.forename: Bart
authors.initials: B
authors.lastname: Meyns
authors.affiliation: Pediatric and Congenital Cardiac Surgery, University Children's Hospital, Zurich, Switzerland.
authors.forename: René
authors.initials: R
authors.lastname: Prêtre
authors.affiliation: Department of Cardiothoracic Surgery, Rigs Hospitalet, Copenhagen, Denmark.
authors.forename: Morten
authors.initials: M
authors.lastname: Helvind
authors.affiliation: Clinic for Cardiovascular Surgery, Swiss Cardiovascular Center University Hospital, Bern, Switzerland.
authors.forename: Thierry
authors.initials: T
authors.lastname: Carrel
authors.affiliation: Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, Netherlands.
authors.forename: Tjark
authors.initials: T
authors.lastname: Ebels
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000706:statistics & numerical data*; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000601:surgery; D006333:Heart Failure; D016027:Heart Transplantation / Q000401:mortality*; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Congenital heart disease; Failing Fontan; Fontan operation; Heart failure; Heart transplantation