title: How good is a good Fontan? Quality of life and exercise capacity of Fontans without arrhythmias.
abstract: BACKGROUND
  Poor long-term outcomes are expected after Fontan surgery, but these perspectives have been tainted by the poorly functioning Fontans suffering from arrhythmias. No predictions of outcome can be quoted to the increasing number of Fontan patients free from arrhythmic complications. The parameters determining improved exercise capacity and quality of life in this subgroup are yet unknown.
  
  
  METHODS
  Fontan survivors from our institution and living in Victoria were invited to participate in the study if they were more than 10 years of age, and free of arrhythmias. A mean of 17 +/- 4 years after Fontan, 36 patients, 23 with a classical atriopulmonary connection (AP) and 13 with a lateral tunnel (LT) underwent transthoracic echocardiography, cycloergometer exercise study, neurohumoral screening, and assessment of quality of life.
  
  
  RESULTS
  The only factor predicting worse exercise capacity was the type of Fontan performed; patients with LT having better exercise capacity than those with AP (percentage of predicted anaerobic threshold: 88 +/- 14% vs 72 +/- 14%, p < 0.005; percentage of predicted VO(2)max: 62 +/- 8% vs 54 +/- 7%, p < 0.005). Endothelin-1 levels were elevated in all patients (2.9 pmol/L, 2.5 to 3.7). Responses from the quality of life measures placed our Fontan cohort mainly within the normal population range. None of the preoperative and postoperative variables adversely affected patients' quality of life.
  
  
  CONCLUSIONS
  The anaerobic threshold of arrhythmia-free Fontan patients operated with the lateral tunnel technique was relatively preserved. Despite restricted exercise capacity, Fontan patients, provided that they are free of arrhythmias, have a normal quality of life reflected in their reports of psychiatric symptoms and family relationships.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Parkville, Victoria, Australia. yves.dudekem@rch.org.au
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
authors.forename: Michael M H
authors.initials: MM
authors.lastname: Cheung
authors.forename: Stella
authors.initials: S
authors.lastname: Setyapranata
authors.forename: Ajay J
authors.initials: AJ
authors.lastname: Iyengar
authors.forename: Patricia
authors.initials: P
authors.lastname: Kelly
authors.forename: Naomi
authors.initials: N
authors.lastname: Buckland
authors.forename: Leeanne E
authors.initials: LE
authors.lastname: Grigg
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.forename: Alasdair
authors.initials: A
authors.lastname: Vance
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.forename: Dan J
authors.initials: DJ
authors.lastname: Penny
mesh_terms: D000293:Adolescent; D001145:Arrhythmias, Cardiac / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D017079:Exercise Tolerance / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D006801:Humans; D015994:Incidence; D008297:Male; D011184:Postoperative Period; D011788:Quality of Life; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014739:Victoria / Q000453:epidemiology; D055815:Young Adult