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

• Published in: Cardiology in the young 2014-06, Vol.24 (3), p.469-477
• Main Authors: McCrindle, Brian W., Zak, Victor, Pemberton, Victoria L., Lambert, Linda M., Vetter, Victoria L., Lai, Wyman W., Uzark, Karen, Margossian, Renee, Atz, Andrew M., Cook, Amanda, Newburger, Jane W.
• Format: Article
• Language: English
• Subjects: Adolescent; Child; Cross-Sectional Studies; Female; Fontan Procedure; Health Status; Heart Defects, Congenital - surgery; Humans; Male; Original Articles; Surveys and Questionnaires
• ISSN: 1047-9511; 1467-1107
• DOI: 10.1017/S1047951113000632

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