title: Functional health status in children and adolescents after Fontan: comparison of generic and disease-specific assessments.
abstract: OBJECTIVE
  The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease.
  
  
  METHODS
  This was a cross-sectional observational study involving 325 single ventricle patients, aged 10-18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables were compared.
  
  
  RESULTS
  From the generic assessment, patients perceived marginally lower physical functioning (p = 0.05) but greater freedom from bodily pain compared with a normal population (p < 0.001). The equivalent physical functioning/limitations domain of the generic instrument, compared with the disease-specific instrument, had similar associations (higher multi-variable model R²) with medical history variables (R² = 0.14 versus R² = 0.12, respectively) and stronger associations with exercise testing variables (R² = 0.22 versus R² = 0.06). Similarly, the corresponding freedom from bodily pain/symptoms domains from both questionnaires showed a greater association for the generic instrument with medical history variables (R² = 0.15 versus R² = 0.09, respectively) and non-cardiac conditions (R² = 0.13 versus R² = 0.06). The associations of each questionnaire with echocardiographic results, cardiac magnetic resonance imaging results, and serum brain natriuretic peptide levels were uniformly weak (R² range <0.01 to 0.04).
  
  
  CONCLUSIONS
  Assessment of the physical functional health status using generic and disease-specific instruments yields few differences with regard to associations between conceptually similar domains and patient and clinical characteristics for adolescents after Fontan procedure.
authors.affiliation: 1 The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
authors.affiliation: 2 New England Research Institutes, Watertown, Massachusetts.
authors.forename: Victor
authors.initials: V
authors.lastname: Zak
authors.affiliation: 3 National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Pemberton
authors.affiliation: 4 Primary Children's Medical Center, University of Utah, Salt Lake City, Utah.
authors.forename: Linda M
authors.initials: LM
authors.lastname: Lambert
authors.affiliation: 5 The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Vetter
authors.affiliation: 6 Columbia University Medical Center, New York, New York.
authors.forename: Wyman W
authors.initials: WW
authors.lastname: Lai
authors.affiliation: 7 Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Karen
authors.initials: K
authors.lastname: Uzark
authors.affiliation: 8 Children's Hospital Boston, Boston, Massachusetts.
authors.forename: Renee
authors.initials: R
authors.lastname: Margossian
authors.affiliation: 9 Medical University of South Carolina, Charleston, South Carolina.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Atz
authors.affiliation: 10 Wake Forest University Baptist Medical Center, Winston-Salem, North Carolina.
authors.forename: Amanda
authors.initials: A
authors.lastname: Cook
authors.affiliation: 8 Children's Hospital Boston, Boston, Massachusetts.
authors.forename: Jane W
authors.initials: JW
authors.lastname: Newburger
mesh_terms: D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D018729:Fontan Procedure; D006304:Health Status; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008297:Male; D011795:Surveys and Questionnaires