title: Does functional health status predict health-related quality of life in children after Fontan operation?
abstract: OBJECTIVE
  It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status - medical history and present medical status - on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents.
  
  
  METHODS
  We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years. Functional health status was assessed as medical history - that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures - and present medical status - assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form.
  
  
  RESULTS
  In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains.
  
  
  CONCLUSIONS
  Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life.
authors.affiliation: 1Department of Child and Adolescent Psychiatry/Psychology,Erasmus Medical Centre-Sophia Children's Hospital,Rotterdam,the Netherlands.
authors.forename: Karolijn
authors.initials: K
authors.lastname: Dulfer
authors.affiliation: 2Department of Paediatrics,Division of Cardiology,Erasmus Medical Centre - Sophia Children's Hospital,Rotterdam,the Netherlands.
authors.forename: Sjoerd S M
authors.initials: SS
authors.lastname: Bossers
authors.affiliation: 1Department of Child and Adolescent Psychiatry/Psychology,Erasmus Medical Centre-Sophia Children's Hospital,Rotterdam,the Netherlands.
authors.forename: Elisabeth M W J
authors.initials: EM
authors.lastname: Utens
authors.affiliation: 2Department of Paediatrics,Division of Cardiology,Erasmus Medical Centre - Sophia Children's Hospital,Rotterdam,the Netherlands.
authors.forename: Nienke
authors.initials: N
authors.lastname: Duppen
authors.affiliation: 3Department of Paediatrics,Division of Cardiology,Academic Medical Centre,Amsterdam,the Netherlands.
authors.forename: Irene M
authors.initials: IM
authors.lastname: Kuipers
authors.affiliation: 4Department of Paediatrics,Division of Cardiology,Radboud University Nijmegen Medical Centre,Nijmegen,the Netherlands.
authors.forename: Livia
authors.initials: L
authors.lastname: Kapusta
authors.affiliation: 6Department of Paediatrics,Division of Cardiology,University Medical Centre Utrecht - Wilhelmina Children's Hospital,Utrecht,the Netherlands.
authors.forename: Gabrielle
authors.initials: G
authors.lastname: van Iperen
authors.affiliation: 4Department of Paediatrics,Division of Cardiology,Radboud University Nijmegen Medical Centre,Nijmegen,the Netherlands.
authors.forename: Michiel
authors.initials: M
authors.lastname: Schokking
authors.affiliation: 7Department of Paediatrics,Division of Cardiology,Leiden University Medical Centre,Leiden,the Netherlands.
authors.forename: Arend D J
authors.initials: AD
authors.lastname: ten Harkel
authors.affiliation: 8Child Development and Exercise Centre,University Medical Centre Utrecht - Wilhelmina Children's Hospital,Utrecht,the Netherlands.
authors.forename: Tim
authors.initials: T
authors.lastname: Takken
authors.affiliation: 2Department of Paediatrics,Division of Cardiology,Erasmus Medical Centre - Sophia Children's Hospital,Rotterdam,the Netherlands.
authors.forename: Willem A
authors.initials: WA
authors.lastname: Helbing
mesh_terms: D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D005080:Exercise Test; D005260:Female; D018729:Fontan Procedure; D006304:Health Status; D006330:Heart Defects, Congenital / Q000601:surgery*; D006352:Heart Ventricles / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D015999:Multivariate Analysis; D009426:Netherlands; D010290:Parents; D011446:Prospective Studies; D011788:Quality of Life; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
keywords: Fontan circulation; congenital heart disease; quality of life