title: Factors associated with long-term mortality after Fontan procedures: a systematic review.
abstract: Despite an ageing Fontan population, data on late outcomes are still scarce. Reported outcome measures and determinants vary greatly between studies making comprehensive appraisal of mortality hazard challenging.
  
  
  
  We conducted a systematic review to evaluate causes and factors associated with late mortality in patients with Fontan circulation. Late mortality was defined as mortality beyond the first postoperative year. Studies were included if they had ≥90 patients or ≥20 late mortalities and/or transplants. Studies with overlapping patients were rationalised to include only the most recent studies to avoid duplication.
  
  
  
  From 28 studies, a total of 6707 patients with an average follow-up time of 8.23±5.42 years was identified. There were 1000 deaths. Causes of late death were reported in 697 cases. The five most common causes were heart/Fontan failure (22%), arrhythmia (16%), respiratory failure (15%), renal disease (12%) and thrombosis/bleeding (10%). Factors associated with late mortality were evaluated and classified into 9 categories.
  
  
  
  Causes and factors associated with late mortality after the Fontan operation are summarised in this study. The presented information will aid in identifying patients at highest risk for mortality and guide our risk stratification efforts in this patient population.
authors.affiliation: Children's Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Tarek
authors.initials: T
authors.lastname: Alsaied
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.|The Netherlands Interuniversity Cardiology Institute of the Netherlands-Netherlands Heart Institute, Utrecht, The Netherlands.
authors.forename: Jouke P
authors.initials: JP
authors.lastname: Bokma
authors.affiliation: Department of Medicine, Groote Schuur Hospital, Cape Town, South Africa.
authors.forename: Mark E
authors.initials: ME
authors.lastname: Engel
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.|The Netherlands Interuniversity Cardiology Institute of the Netherlands-Netherlands Heart Institute, Utrecht, The Netherlands.
authors.forename: Joey M
authors.initials: JM
authors.lastname: Kuijpers
authors.affiliation: Children's Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Samuel P
authors.initials: SP
authors.lastname: Hanke
authors.affiliation: Department of Paediatrics, Red Cross War Memorial Children's Hospital and University of Cape Town, Cape Town, South Africa.
authors.forename: Liesl
authors.initials: L
authors.lastname: Zuhlke
authors.affiliation: Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio USA.
authors.forename: Bin
authors.initials: B
authors.lastname: Zhang
authors.affiliation: Adolescent and Adult Congenital Program, Children's Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Gruschen R
authors.initials: GR
authors.lastname: Veldtman
mesh_terms: D002423:Cause of Death; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery; D006801:Humans; D011379:Prognosis; D012307:Risk Factors; D016896:Treatment Outcome