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Psychosocial adjustment and quality of life in children undergoing screening in a specialist paediatric hypertrophic cardiomyopathy clinic

Published online by Cambridge University Press:  08 September 2015

Adriani Spanaki ,
Sara O’Curry ,
Jasmine Winter-Beatty ,
Sarah Mead-Regan ,
Kate Hawkins ,
Jennifer English ,
Catherine Head ,
Deborah Ridout ,
Maria T. Tome-Esteban  and
Perry Elliott
...Show all authors
Adriani Spanaki
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom
Sara O’Curry
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom Department of Clinical Psychology, Great Ormond Street Hospital, London, United Kingdom
Jasmine Winter-Beatty
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom King’s College Medical School, London, United Kingdom
Sarah Mead-Regan
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom
Kate Hawkins
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom Department of Clinical Psychology, Great Ormond Street Hospital, London, United Kingdom
Jennifer English
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom Department of Clinical Psychology, Great Ormond Street Hospital, London, United Kingdom
Catherine Head
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom Institute of Cardiovascular Science, University College London, London, United Kingdom
Deborah Ridout
Affiliation:
Pediatric Epidemiology Unit, Institute of Child Health, London, United Kingdom
Maria T. Tome-Esteban
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom
Perry Elliott
Affiliation:
Institute of Cardiovascular Science, University College London, London, United Kingdom
Juan P. Kaski*
Affiliation:
Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, United Kingdom Institute of Cardiovascular Science, University College London, London, United Kingdom
*
Correspondence to: Dr J. P. Kaski, Department of Cardiology, Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London WC1N 3JH, United Kingdom. Tel: +44 207 829 8839; Fax: +44 207 829 8673; E-mail: [email protected]
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Abstract

Objective

This study aimed to assess the psychological well-being and quality of life in children with hypertrophic cardiomyopathy and the potential psychosocial impact of screening.

Methods

A total of 152 children (aged 3–18 years) attending a specialist paediatric hypertrophic cardiomyopathy clinic, and their parents completed the Generic Core Scales and Cardiac Module of the Paediatric Quality of Life Inventory (PedsQL) questionnaire as well as the Strengths and Difficulties Questionnaire; 21 patients (14%) had hypertrophic cardiomyopathy (group A); 23 children (15%) harboured hypertrophic cardiomyopathy-causing sarcomeric mutations with normal echocardiograms (group G); and 108 children (71%) had a family history of hypertrophic cardiomyopathy with normal investigations and attended for clinical cardiological screening (group S).

Results

In group A, mean PedsQLTM total scores reported by children and parents were lower than those reported by unaffected children (p<0.001). There was no significant difference between unaffected and gene-positive patients. Mean Cardiac module PedsQLTM total scores by children and parents were lower in children with hypertrophic cardiomyopathy compared with unaffected patients [mean child-reported total score 86.4 in group S versus 72.3 in group A (p<0.001) and 80.2 in group G (p=0.25); mean parent-reported total score 91.6 in group S versus 71.4 in group A (p<0.001) and 87 in group G (p=0.4)]. There was no significant difference between group S and group G on any of the scales, or between the three groups of patients in the mean Strengths and Difficulties Questionnaire scores.

Conclusions

Children with hypertrophic cardiomyopathy have a significantly reduced quality of life. Importantly, Quality-of-Life scores among unaffected children attending for screening were not different compared with scores from a normative UK population.

Keywords

Quality of lifehypertrophic cardiomyopathyscreeningchildren
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 5 , June 2016 , pp. 961 - 967
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Copyright
© Cambridge University Press 2015 

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References

1. Elliott, P, Andersson, B, Arbustini, E, et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2008; 29: 270–276.CrossRefGoogle Scholar
2. Moak, JP, Kaski, JP. Hypertrophic cardiomyopathy in children. Heart 2012; 98: 1044–1054.Google Scholar
3. Bratt, EL, Ostman-Smith, I, Sparud-Lundin, C, Axelsson, BA. Parents’ experiences of having an asymptomatic child diagnosed with hypertrophic cardiomyopathy through family screening. Cardiol Young 2011; 21: 8–14.Google Scholar
4. Kaski, JP, Syrris, P, Esteban, MT, et al. Prevalence of sarcomere protein gene mutations in preadolescent children with hypertrophic cardiomyopathy. Circ Cardiovasc Genet 2009; 2: 436–441.Google Scholar
5. Cox, S, O’Donoghue, AC, McKenna, WJ, Steptoe, A. Health related quality of life and psychological wellbeing in patients with hypertrophic cardiomyopathy. Heart 1997; 78: 182–187.Google Scholar
6. Bratt, EL, Ostman-Smith, I, Axelsson, A, Berntsson, L. Quality of life in asymptomatic children and adolescents before and after diagnosis of hypertrophic cardiomyopathy through family screening. J Clin Nurs 2013; 22: 211–221.Google Scholar
7. Bratt, EL, Sparud-Lundin, C, Ostman-Smith, I, Axelsson, AB. The experience of being diagnosed with hypertrophic cardiomyopathy through family screening in childhood and adolescence. Cardiol Young 2012; 22: 528–535.Google Scholar
8. Aatre, RD, Day, SM. Psychological issues in genetic testing for inherited cardiovascular diseases. Circ Cardiovasc Genet 2011; 4: 81–90.CrossRefGoogle ScholarPubMed
9. 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
10. Varni, JW, Seid, M, Kurtin, PS. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care 2001; 39: 800–812.CrossRefGoogle ScholarPubMed
11. Goodman, R. Psychometric properties of the strengths and difficulties questionnaire. J Am Acad Child Adolesc Psychiatry 2001; 40: 1337–1345.CrossRefGoogle ScholarPubMed
12. Ingles, J, Yeates, L, O’Brien, L, et al. Genetic testing for inherited heart diseases: longitudinal impact on health-related quality of life. Genet Med 2012; 14: 749–752.Google Scholar
13. Ingles, J, Lind, JM, Phongsavan, P, Semsarian, C. Psychosocial impact of specialized cardiac genetic clinics for hypertrophic cardiomyopathy. Genet Med 2008; 10: 117–120.Google Scholar
14. Morgan, JF, O’Donoghue, AC, McKenna, WJ, Schmidt, MM. Psychiatric disorders in hypertrophic cardiomyopathy. Gen Hosp Psychiatry 2008; 30: 49–54.CrossRefGoogle ScholarPubMed
15. Smets, EM, Stam, MM, Meulenkamp, TM, et al. Health-related quality of life of children with a positive carrier status for inherited cardiovascular diseases. Am J Med Genet A 2008; 146A: 700–707.Google Scholar
16. Authors/Task Force members, Elliott, PM, Anastasakis, A, et al. 2014 ESC guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 2014; 35: 2733–2779.Google Scholar
17. Morita, H, Rehm, HL, Menesses, A, et al. Shared genetic causes of cardiac hypertrophy in children and adults. N Engl J Med 2008; 358: 1899–1908.Google Scholar
18. Ostman-Smith, I, Wettrell, G, Keeton, B, et al. Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy. Eur Heart J 2008; 29: 1160–1167.Google Scholar
19. Charron, P, Arad, M, Arbustini, E, et al. Genetic counselling and testing in cardiomyopathies: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2010; 31: 2715–2726.Google Scholar