Psychosocial adjustment and quality of life in children undergoing screening in a specialist paediatric hypertrophic cardiomyopathy clinic.
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.
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Adriani
A
Spanaki
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Sara
S
O'Curry
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Jasmine
J
Winter-Beatty
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Sarah
S
Mead-Regan
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Kate
K
Hawkins
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Jennifer
J
English
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Catherine
C
Head
4Pediatric Epidemiology Unit,Institute of Child Health,London,United Kingdom.
Deborah
D
Ridout
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Maria T
MT
Tome-Esteban
5Institute of Cardiovascular Science,University College London,London,United Kingdom.
Perry
P
Elliott
1Department of Cardiology,Inherited Cardiovascular Diseases Unit,Great Ormond Street Hospital,London,United Kingdom.
Juan P
JP
Kaski
D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000523:psychology*; D002648:Child; D005260:Female; D006304:Health Status; D006801:Humans; D008131:London; D008297:Male; D010290:Parents / Q000523:psychology*; D011569:Psychiatric Status Rating Scales; D011788:Quality of Life; D012044:Regression Analysis; D011795:Surveys and Questionnaires
Quality of life; children; hypertrophic cardiomyopathy; screening