title: Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation.
abstract: OBJECTIVE
  To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan.
  
  
  METHODS
  Cross-sectional. The Pediatric Quality of Life Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age <19 years) or Short Form Health Survey (age ≥ 19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined.
  
  
  RESULTS
  Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL (P < .001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points (P = .004) and the emotional functioning score decreased by an average of 0.64 points (P = .03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age (P = .02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire (P < .001) and Short Form Health Survey scores (P < .001).
  
  
  CONCLUSIONS
  Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood.
  
  
  BACKGROUND
  ClinicalTrials.gov: NCT00132782.
authors.affiliation: University of Michigan Mott Children's Hospital, Ann Arbor, MI. Electronic address: karenu@med.umich.edu.
authors.forename: Karen
authors.initials: K
authors.lastname: Uzark
authors.affiliation: New England Research Institutes, Watertown, MA.
authors.forename: Victor
authors.initials: V
authors.lastname: Zak
authors.affiliation: New England Research Institutes, Watertown, MA.
authors.forename: Peter
authors.initials: P
authors.lastname: Shrader
authors.affiliation: University of Toronto Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
authors.affiliation: University of Toronto Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Radojewski
authors.affiliation: Texas A & M University, College Station, TX.
authors.forename: James W
authors.initials: JW
authors.lastname: Varni
authors.affiliation: Children's Hospital of Philadelphia, Philadelphia, PA.
authors.forename: Kaitlyn
authors.initials: K
authors.lastname: Daniels
authors.affiliation: Boston Children's Hospital, Boston, MA.
authors.forename: Jill
authors.initials: J
authors.lastname: Handisides
authors.affiliation: Duke University Medical Center, Durham, NC.
authors.forename: Kevin D
authors.initials: KD
authors.lastname: Hill
authors.affiliation: Primary Children's Hospital, Salt Lake City, UT.
authors.forename: Linda M
authors.initials: LM
authors.lastname: Lambert
authors.affiliation: Boston Children's Hospital, Boston, MA.
authors.forename: Renee
authors.initials: R
authors.lastname: Margossian
authors.affiliation: National Heart, Lung, and Blood Institute, Bethesda, MD.
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Pemberton
authors.affiliation: Columbia University Medical Center, New York, NY.
authors.forename: Wyman W
authors.initials: WW
authors.lastname: Lai
authors.affiliation: Medical University of South Carolina, Charleston, SC.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Atz
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D003430:Cross-Sectional Studies; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000523:psychology*; D006801:Humans; D008297:Male; D011788:Quality of Life; D011795:Surveys and Questionnaires; D017741:Survivors; D014481:United States / Q000453:epidemiology; D055815:Young Adult