title: Health-Related Quality of Life and Functional Status Are Associated with Cardiac Status and Clinical Outcome in Children with Cardiomyopathy.
abstract: OBJECTIVE
  To measure the health-related quality of life (HRQOL) and functional status of children with cardiomyopathy and to determine whether they are correlated with sociodemographics, cardiac status, and clinical outcomes.
  
  
  METHODS
  Parents of children in the Pediatric Cardiomyopathy Registry completed the Child Health Questionnaire (CHQ; age ≥ 5 years) and Functional Status II (Revised) (age ≤ 18 years) instruments. Linear and Cox regressions were used to examine hypothesized associations with HRQOL.
  
  
  RESULTS
  The 355 children evaluated at ≥ 5 years (median 8.6 years) had lower functioning (CHQ Physical and Psychosocial Summary Scores 41.7 ± 14.4 and 47.8 ± 10.7) than that of healthy historical controls. The most extreme CHQ domain score, Parental Impact-Emotional, was one SD below normal. Younger age at diagnosis and smaller left ventricular end-diastolic dimension z score were associated independently with better physical functioning in children with dilated cardiomyopathy. Greater income/education correlated with better psychosocial functioning in children with hypertrophic and mixed/other types of cardiomyopathy. In the age ≥ 5 year cohort, lower scores on both instruments predicted earlier death/transplant and listing for transplant in children with dilated and mixed/other types of cardiomyopathy (P < .001). Across all ages (n = 565), the Functional Status II (Revised) total score was 87.1 ± 16.4, and a lower score was associated with earlier death/transplant for all cardiomyopathies.
  
  
  CONCLUSIONS
  HRQOL and functional status in children with cardiomyopathy is on average impaired relative to healthy children. These impairments are associated with older age at diagnosis, lower socioeconomic status, left ventricular size, and increased risk for death and transplant. Identification of families at risk for functional impairment allows for provision of specialized services early in the course of disease.
  
  
  BACKGROUND
  ClinicalTrials.gov: NCT00005391.
authors.affiliation: New England Research Institutes, Watertown, MA. Electronic address: lynn.sleeper@cardio.chboston.org.
authors.forename: Lynn A
authors.initials: LA
authors.lastname: Sleeper
authors.affiliation: University of Tennessee Health Science Center, St. Jude Children's Research Hospital and Le Bonheur Children's Hospital, Memphis, TN.
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Towbin
authors.affiliation: Boston Children's Hospital, Boston, MA.
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.affiliation: The Children's Hospital at Montefiore/Albert Einstein College of Medicine, Bronx, NY.
authors.forename: Daphne
authors.initials: D
authors.lastname: Hsu
authors.affiliation: Department of Medicine, Harvard Medical School, Boston, MA.
authors.forename: Endel J
authors.initials: EJ
authors.lastname: Orav
authors.affiliation: University of Texas Southwestern Medical Center, Dallas, TX.
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Lemler
authors.affiliation: Texas Children's Hospital, Houston, TX.
authors.forename: Sarah
authors.initials: S
authors.lastname: Clunie
authors.affiliation: Boston Children's Hospital, Boston, MA.
authors.forename: Jane
authors.initials: J
authors.lastname: Messere
authors.affiliation: Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, TN.
authors.forename: Darlene
authors.initials: D
authors.lastname: Fountain
authors.affiliation: University of Miami Miller School of Medicine, Miami, FL.
authors.forename: Tracie L
authors.initials: TL
authors.lastname: Miller
authors.affiliation: Department of Pediatrics, Wayne State University School of Medicine and Children's Hospital of Michigan, Detroit, MI.
authors.forename: James D
authors.initials: JD
authors.lastname: Wilkinson
authors.affiliation: Department of Pediatrics, Wayne State University School of Medicine and Children's Hospital of Michigan, Detroit, MI.
authors.forename: Steven E
authors.initials: SE
authors.lastname: Lipshultz
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002311:Cardiomyopathy, Dilated / Q000453:epidemiology* / Q000601:surgery; D002312:Cardiomyopathy, Hypertrophic / Q000453:epidemiology* / Q000601:surgery; D002648:Child; D004522:Educational Status; D005260:Female; D016027:Heart Transplantation / Q000706:statistics & numerical data; D006801:Humans; D007182:Income; D008297:Male; D015999:Multivariate Analysis; D011788:Quality of Life; D012042:Registries; D014481:United States / Q000453:epidemiology