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Functional health status in children and adolescents after Fontan: comparison of generic and disease-specific assessments

Published online by Cambridge University Press:  10 June 2013

Brian W. McCrindle*
The Hospital for Sick Children, University of Toronto, Toronto, Canada
Victor Zak
New England Research Institutes, Watertown, Massachusetts
Victoria L. Pemberton
National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland
Linda M. Lambert
Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
Victoria L. Vetter
The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
Wyman W. Lai
Columbia University Medical Center, New York, New York
Karen Uzark
Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Renee Margossian
Children's Hospital Boston, Boston, Massachusetts
Andrew M. Atz
Medical University of South Carolina, Charleston, South Carolina
Amanda Cook
Wake Forest University Baptist Medical Center, Winston-Salem, North Carolina
Jane W. Newburger
Children's Hospital Boston, Boston, Massachusetts
Correspondence to: Dr B. McCrindle, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8. Tel: +416 813 7610; Fax: +416 813 7547; E-mail:


Purpose: 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.

Original Articles
Copyright © Cambridge University Press 2013 

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