title: Parent reports of health-related quality of life and heart failure severity score independently predict outcome in children with dilated cardiomyopathy.
abstract: BACKGROUND
  Dilated cardiomyopathy in children causes heart failure and has a poor prognosis. Health-related quality of life in this patient group is unknown. Moreover, results may provide detailed information of parents' sense of their child's functioning. We hypothesised that health-related quality of life, as rated by parents, and the paediatric heart failure score, as assessed by physicians, have both predictive value on outcome. Methods and results In this prospective study, health-related quality of life was assessed by parent reports: the Infant Toddler Quality of Life questionnaire (0-4 years) or Child Health Questionnaire-Parent Form 50 (4-18 years) at 3-6-month intervals. We included 90 children (median age 3.8 years, interquartile range (IQR) 0.9-12.3) whose parents completed 515 questionnaires. At the same visit, physicians completed the New York University Pediatric Heart Failure Index. Compared with Dutch normative data, quality of life was severely impaired at diagnosis (0-4 years: 7/10 subscales and 4-18 years: 8/11 subscales) and ⩾1 year after diagnosis (3/10 and 6/11 subscales). Older children were more impaired (p<0.05). After a median follow-up of 3 years (IQR 2-4), 15 patients underwent transplantation. Using multivariable time-dependent Cox regression, "physical functioning" subscale and the Heart Failure Index were independently predictive of the risk of death and heart transplantation (hazard ratio 1.24 per 10% decrease of predicted, 95% confidence interval (CI) 1.06-1.47 and hazard ratio 1.38 per unit, 95% CI 1.19-1.61, respectively).
  
  
  CONCLUSIONS
  Physical impairment rated by parents and heart failure severity assessed by physicians independently predicted the risk of death or heart transplantation in children with dilated cardiomyopathy.
authors.affiliation: 1Department of Pediatrics,Division of Pediatric Cardiology,Sophia Children's Hospital,Rotterdam,The Netherlands.
authors.forename: Susanna L
authors.initials: SL
authors.lastname: den Boer
authors.affiliation: 2Department of Cardiology,Erasmus University Medical Center,Rotterdam,Erasmus University Medical Center,Rotterdam,The Netherlands.
authors.forename: Sara J
authors.initials: SJ
authors.lastname: Baart
authors.affiliation: 1Department of Pediatrics,Division of Pediatric Cardiology,Sophia Children's Hospital,Rotterdam,The Netherlands.
authors.forename: Marijke H
authors.initials: MH
authors.lastname: van der Meulen
authors.affiliation: 3Department of Pediatrics,Division of Pediatric Cardiology,Wilhelmina Children's Hospital,University Medical Center Utrecht,Utrecht,The Netherlands.
authors.forename: Gabriëlle G
authors.initials: GG
authors.lastname: van Iperen
authors.affiliation: 4Department of Pediatrics,Division of Pediatric Cardiology,Emma Children's Hospital,Academic Medical Center,Amsterdam,The Netherlands.
authors.forename: Ad P
authors.initials: AP
authors.lastname: Backx
authors.affiliation: 5Department of Pediatrics,Division of Pediatric Cardiology,Leiden University Medical Center,Leiden,The Netherlands.
authors.forename: Arend D
authors.initials: AD
authors.lastname: Ten Harkel
authors.affiliation: 6Department of Pediatrics,Division of Pediatric Cardiology,Free University Medical Center,Amsterdam,Amsterdam.
authors.forename: Lukas A
authors.initials: LA
authors.lastname: Rammeloo
authors.affiliation: 7Department of Pediatrics,Division of Pediatric Cardiology, Beatrix Children's Hospital,University of Groningen, Groningen,The Netherlands.
authors.forename: Gideon J
authors.initials: GJ
authors.lastname: du Marchie Sarvaas
authors.affiliation: 8Department of Pediatrics, Division of Pediatric,Cardiology,RadboudUniversity Medical Center,Nijmegen,The Netherlands.
authors.forename: Ronald B
authors.initials: RB
authors.lastname: Tanke
authors.affiliation: 1Department of Pediatrics,Division of Pediatric Cardiology,Sophia Children's Hospital,Rotterdam,The Netherlands.
authors.forename: Willem A
authors.initials: WA
authors.lastname: Helbing
authors.affiliation: 9Department of Child and Adolescent Psychiatry/Psychology,Sophia Children's Hospital,Erasmus University Medical Center,Rotterdam,The Netherlands.
authors.forename: Elisabeth M
authors.initials: EM
authors.lastname: Utens
authors.affiliation: 1Department of Pediatrics,Division of Pediatric Cardiology,Sophia Children's Hospital,Rotterdam,The Netherlands.
authors.forename: Michiel
authors.initials: M
authors.lastname: Dalinghaus
mesh_terms: D000293:Adolescent; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000175:diagnosis / Q000523:psychology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006304:Health Status; D006333:Heart Failure / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D010290:Parents; D011446:Prospective Studies; D011788:Quality of Life; D012042:Registries; D018570:Risk Assessment / Q000379:methods*; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires
keywords: New York University Pediatric Heart Failure Index; Quality of life; children; dilated cardiomyopathy; outcome