title: Functional health status of adolescents after the Fontan procedure -- comparison with their siblings.
abstract: BACKGROUND
  Studies have suggested that patients who have undergone the Fontan procedure experience lower functional health status and diminished exercise capacity compared with other children.
  
  
  OBJECTIVE
  To compare the functional health status of Fontan patients with and without siblings, assess whether there are any differences between Fontan patients and their siblings, and determine associated factors.
  
  
  METHODS
  A cross-sectional, single-centre, observational study was performed on Fontan patients 10 to 20 years of age, and their sibling closest in age, followed in a tertiary pediatric hospital. Functional health status was measured by the Child Health Questionnaire Child Form and the Pediatric Quality of Life Inventory.
  
  
  RESULTS
  A total of 68 patients and 38 siblings were enrolled. Patients with siblings scored significantly lower on numerous domains of physical functional status than those without siblings. Compared with their matched siblings, Fontan patients reported significantly lower scores in all domains of the Pediatric Quality of Life Inventory and on physical (but not psychosocial) domains of the Child Health Questionnaire Child Form. Factors associated with increased patient-sibling differences included younger patient age, female sex, intracardiac lateral tunnel Fontan connection and lower ejection fraction at the time of study enrollment.
  
  
  CONCLUSIONS
  Adolescents with Fontan physiology reported a lower functional health status in physical domains than their siblings, but had similar status in psychosocial domains. Having a sibling was associated with lower reported functional health status, suggesting an important effect of self-perceived physical limitations over true limitations.
authors.affiliation: Division of Cardiology, Labatt Family Heart Centre, Toronto, Canada.
authors.forename: Cedric
authors.initials: C
authors.lastname: Manlhiot
authors.forename: Stevan
authors.initials: S
authors.lastname: Knezevich
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Radojewski
authors.forename: Geraldine
authors.initials: G
authors.lastname: Cullen-Dean
authors.forename: William G
authors.initials: WG
authors.lastname: Williams
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
mesh_terms: D000203:Activities of Daily Living; D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D018729:Fontan Procedure / Q000534:rehabilitation*; D006305:Health Status Indicators; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000523:psychology / Q000601:surgery*; D006801:Humans; D008297:Male; D009043:Motor Activity / Q000502:physiology*; D009864:Ontario; D011184:Postoperative Period; D011788:Quality of Life; D035781:Siblings; D011795:Surveys and Questionnaires; D055815:Young Adult