title: Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure.
abstract: BACKGROUND
  Patients after the Fontan procedure are at risk for suboptimal functional health status, and associations with laboratory measures are important for planning interventions and outcome measures for clinical trials.
  
  
  RESULTS
  Parents completed the generic Child Health Questionnaire for 511 Fontan Cross-Sectional Study patients 6 to 18 years of age (61% male). Associations of Child Health Questionnaire Physical and Psychosocial Functioning Summary Scores (FSS) with standardized measurements from prospective exercise testing, echocardiography, magnetic resonance imaging, and measurement of brain natriuretic peptide were determined by regression analyses. For exercise variables for maximal effort patients only, the final model showed that higher Physical FSS was associated only with higher maximum work rate, accounting for 9% of variation in Physical FSS. For echocardiography, lower Tei index (particularly for patients with extracardiac lateral tunnel connections), lower indexed end-systolic volume, and the absence of atrioventricular valve regurgitation for patients having Fontan procedure at age <2 years were associated with higher Physical FSS, accounting for 14% of variation in Physical FSS. For magnetic resonance imaging, ratio of lower mass to end-diastolic volume and midquartiles of indexed end-systolic volume (nonlinear) were associated with higher Physical FSS, accounting for 11% of variation. Lower brain natriuretic peptide was significantly but weakly associated with higher Physical FSS (1% of variation). Significant associations for Psychosocial FSS with laboratory measures were fewer and weaker than for Physical FSS.
  
  
  CONCLUSIONS
  In relatively healthy Fontan patients, laboratory measures account for a small proportion of the variation in functional health status and therefore may not be optimal surrogate end points for trials of therapeutic interventions.
authors.affiliation: Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8. brian.mccrindle@sickkids.ca
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
authors.forename: Victor
authors.initials: V
authors.lastname: Zak
authors.forename: Lynn A
authors.initials: LA
authors.lastname: Sleeper
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Paridon
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.forename: Tal
authors.initials: T
authors.lastname: Geva
authors.forename: Lynn
authors.initials: L
authors.lastname: Mahony
authors.forename: Jennifer S
authors.initials: JS
authors.lastname: Li
authors.forename: Roger E
authors.initials: RE
authors.lastname: Breitbart
authors.forename: Renee
authors.initials: R
authors.lastname: Margossian
authors.forename: Richard V
authors.initials: RV
authors.lastname: Williams
authors.forename: Welton M
authors.initials: WM
authors.lastname: Gersony
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Atz
mesh_terms: D000293:Adolescent; D002306:Cardiac Volume; D002648:Child; D003430:Cross-Sectional Studies; D004452:Echocardiography; D005080:Exercise Test; D017079:Exercise Tolerance / Q000502:physiology*; D005260:Female; D018729:Fontan Procedure; D006304:Health Status; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011183:Postoperative Complications / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D011795:Surveys and Questionnaires; D018754:Ventricular Dysfunction / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*
chemical_list: D020097:Natriuretic Peptide, Brain