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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 ,
Victor Zak ,
Victoria L. Pemberton ,
Linda M. Lambert ,
Victoria L. Vetter ,
Wyman W. Lai ,
Karen Uzark ,
Renee Margossian ,
Andrew M. Atz  and
Amanda Cook
...Show all authors
Brian W. McCrindle*
Affiliation:
The Hospital for Sick Children, University of Toronto, Toronto, Canada
Victor Zak
Affiliation:
New England Research Institutes, Watertown, Massachusetts
Victoria L. Pemberton
Affiliation:
National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland
Linda M. Lambert
Affiliation:
Primary Children's Medical Center, University of Utah, Salt Lake City, Utah
Victoria L. Vetter
Affiliation:
The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
Wyman W. Lai
Affiliation:
Columbia University Medical Center, New York, New York
Karen Uzark
Affiliation:
Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Renee Margossian
Affiliation:
Children's Hospital Boston, Boston, Massachusetts
Andrew M. Atz
Affiliation:
Medical University of South Carolina, Charleston, South Carolina
Amanda Cook
Affiliation:
Wake Forest University Baptist Medical Center, Winston-Salem, North Carolina
Jane W. Newburger
Affiliation:
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: brian.mccrindle@sickkids.ca
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Abstract

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.

Keywords

Fontan procedurecardiac defectscongenitalpaediatrics
Type
Original Articles
Information
Cardiology in the Young , Volume 24 , Issue 3 , June 2014 , pp. 469 - 477
Copyright
Copyright © Cambridge University Press 2013 

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References

1. Freedom, RM, Hamilton, R, Yoo, SJ, et al. The Fontan procedure: analysis of cohorts and late complications. Cardiol Young 2000; 10: 307–331.Google Scholar
2. Sleeper, LA, Anderson, P, Hsu, DT, et al. Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation. Am Heart J 2006; 152: 427–433.Google Scholar
3. Landgraf, JM, Abetz, L, Ware, JE. The Child Health Questionnaire (CHQ) User's Manual, 2nd Printing, HealthAct, Boston, MA, 1999.Google Scholar
4. McCrindle, BW, Williams, RV, Mitchell, PD, et al. Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation 2006; 113: 1123–1129.CrossRefGoogle ScholarPubMed
5. Lambert, LM, Minich, LL, Newburger, JW, et al. Parent- versus child-reported functional health status after the Fontan procedure. Pediatrics 2009; 124: e942–e949.CrossRefGoogle ScholarPubMed
6. Manlhiot, C, Knezevich, S, Radojewski, E, Cullen-Dean, G, Williams, WG, McCrindle, BW. Functional health status of adolescents after the Fontan procedure – comparison with their siblings. Can J Cardiol 2009; 25: e294–e300.Google Scholar
7. McCrindle, BW, Williams, RV, Mital, S, et al. Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health. Arch Dis Child 2007; 92: 509–514.Google Scholar
8. Kendall, L, Lewin, RJ, Parsons, JM, Veldtman, GR, Quirk, J, Hardman, GE. Factors associated with self-perceived state of health in adolescents with congenital cardiac disease attending paediatric cardiologic clinics. Cardiol Young 2001; 11: 431–438.Google Scholar
9. Mahony, L, Sleeper, LA, Anderson, PA, et al. The Pediatric Heart Network, A primer for the conduct of multicenter studies in children with congenital and acquired heart disease. Pediatr Cardiol HealthAct, Boston, MA. 2006; 27: 191–198.CrossRefGoogle ScholarPubMed
10. McCrindle, BW, Zak, V, Sleeper, LA, et al. Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure. Circulation 2010; 121: 34–42.CrossRefGoogle ScholarPubMed
11. Landgraf, JM, Abetz, LN. Functional status and well-being of children representing three cultural groups. Initial self-reports using the CHQ-CF87. Psychol Health 1997; 12: 839–854.Google Scholar
12. Bergner, M. Quality of life, health status, and clinical research. Med Care 1989; 27: S148–S156.Google Scholar
13. Moons, P. Patient-reported outcomes in congenital cardiac disease: are they as good as you think they are? Cardiol Young 2010; 20: 143–148.Google Scholar
14. Moons, P, Van Deyk, K, Budts, W, De Geest, S. Caliber of quality-of-life assessments in congenital heart disease: a plea for more conceptual and methodological rigor. Arch Pediatr Adolesc Med 2004; 158: 1062–1069.Google Scholar
15. Moons, P. Why call it health-related quality of life when you mean perceived health status? Eur J Cardiovasc Nurs 2004; 3: 275–277.Google Scholar
16. Moons, P, Budts, W, De Geest, S. Critique on the conceptualisation of quality of life: a review and evaluation of different conceptual approaches. Int J Nurs Stud 2006; 43: 891–901.Google Scholar
17. Marino, BS, Tomlinson, RS, Drotar, D, et al. Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease. Pediatrics 2009; 123: e708–e715.Google Scholar
18. Arafa, MA, Zaher, SR, El-Dowaty, AA, Moneeb, DE. Quality of life among parents of children with heart disease. Health Qual Life Outcomes 2008; 6: 91.Google Scholar
19. Varni, JW, Limbers, CA, Burwinkle, TM. Parent proxy-report of their children's health-related quality of life: an analysis of 13,878 parents’ reliability and validity across age subgroups using the PedsQL 4.0 Generic Core Scales. Health Qual Life Outcomes 2007; 5: 2.Google Scholar
20. Landolt, MA, Valsangiacomo Buechel, ER, Latal, B. Health-related quality of life in children and adolescents after open-heart surgery. J Pediatr 2008; 152: 349–355.Google Scholar
21. Spijkerboer, AW, Utens, EM, De Koning, WB, Bogers, AJ, Helbing, WA, Verhulst, FC. Health-related quality of life in children and adolescents after invasive treatment for congenital heart disease. Qual Life Res 2006; 15: 663–673.CrossRefGoogle ScholarPubMed
22. Raat, H, Bonsel, GJ, Essink-Bot, ML, Landgraf, JM, Gemke, RJ. Reliability and validity of comprehensive health status measures in children: the Child Health Questionnaire in relation to the Health Utilities Index. J Clin Epidemiol 2002; 55: 67–76.Google Scholar
23. Uzark, K, Jones, K, Slusher, J, Limbers, CA, Burwinkle, TM, Varni, JW. Quality of life in children with heart disease as perceived by children and parents. Pediatrics 2008; 121: e1060–e1067.Google Scholar
24. Uzark, K, Jones, K, Burwinkle, TM, Varni, JW. The pediatric quality of life inventory in children with heart disease. Prog Pediatr Cardiol 2003; 18: 141–148.Google Scholar
25. Marino, BS, Shera, D, Wernovsky, G, et al. The development of the Pediatric Cardiac Quality Of Life Inventory: a quality of life measure for children and adolescents with heart disease. Qual Life Res 2008; 17: 613–626.Google Scholar
26. Macran, S, Birks, Y, Parsons, J, et al. The development of a new measure of quality of life for children with congenital cardiac disease. Cardiol Young 2006; 16: 165–172.Google Scholar
27. Kamphuis, M, Zwinderman, KH, Vogels, T, et al. A cardiac-specific health-related quality of life module for young adults with congenital heart disease: development and validation. Qual Life Res 2004; 13: 735–745.Google Scholar
28. Motl, RW, McAuley, E, Snook, EM, Gliottoni, RC. Does the relationship between physical activity and quality of life differ based on generic versus disease-targeted instruments? Ann Behav Med 2008; 36: 93–99.Google Scholar
29. Brouwer, CN, Schilder, AG, van Stel, HF, et al. Reliability and validity of functional health status and health-related quality of life questionnaires in children with recurrent acute otitis media. Qual Life Res 2007; 16: 1357–1373.CrossRefGoogle ScholarPubMed
30. Shi, HY, Lee, HH, Chiu, CC, Chiu, HC, Uen, YH, Lee, KT. Responsiveness and minimal clinically important differences after cholecystectomy: GIQLI versus SF-36. J Gastrointest Surg 2008; 12: 1275–1282.CrossRefGoogle ScholarPubMed
31. Bessette, L, Sangha, O, Kuntz, KM, et al. Comparative responsiveness of generic versus disease-specific and weighted versus unweighted health status measures in carpal tunnel syndrome. Med Care 1998; 36: 491–502.Google Scholar
32. Wolinsky, FD, Wyrwich, KW, Nienaber, NA, Tierney, WM. Generic versus disease-specific health status measures. An example using coronary artery disease and congestive heart failure patients. Eval Health Prof 1998; 21: 216–243.Google Scholar
33. Huang, IC, Hwang, CC, Wu, MY, Lin, W, Leite, W, Wu, AW. Diabetes-specific or generic measures for health-related quality of life? Evidence from psychometric validation of the D-39 and DF-36. Value Health 2008; 11: 450–461.Google Scholar
34. Marino, BS, Tomlinson, RS, Wernovsky, G, et al. Validation of the Pediatric Cardiac Quality Of Life Inventory. Pediatrics 2010; 126: 498–508.Google Scholar
35. Marino, BS, Drotar, D, Cassedy, A, et al. External validity of the pediatric cardiac quality of life inventory. Qual Life Res 2011; 20: 205–214.Google Scholar
Supplementary material: File

McCrindle Supplementary Material

Acknowledgements

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Supplementary material: File

McCrindle Supplementary Material

Table S1

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