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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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