HCM in children: Age Specific risk stratification for sudden death
Research type
Research Study
Full title
Hypertrophic Cardiomyopathy in children: Age specific risk stratification for sudden death
IRAS ID
184951
Contact name
Maria Ilina
Contact email
Sponsor organisation
NHS Greater Glasgow and Clyde
Duration of Study in the UK
0 years, 4 months, 29 days
Research summary
Hypertrophic cardiomyopathy (HCM) is a condition which affects heart muscle and frequently runs in the family. It may become apparent at different ages and have a diverse clinical course. Several disease-causing mutations in genes encoding proteins of the cardiac muscle work unit (sarcomere) have been reported. HCM is a relatively common disorder, occurring in about 1 in 500 of the general population. HCM is the commonest cause of sudden cardiac death (SD) in young athletes and is an important cause of SD in the adolescent and young adult age. While it does not go away when this age is passed, the risk of SD seems to lessen after the age of 35. The objectives of this study are to determine whether or not there are risk factors related to clinical history, family history, ECG, Echocardiogram, MRI, Holter monitoring, or exercise testing results that predict SD in HCM. This is a multi-center, retrospective data collection study. Included will be anonymised data collected from the clinical record of children and adolescents aged 20 years or younger at the time of diagnosis with HCM, by retrospective review of medical records created between January 1, 1980 and January 31, 2013. The clinical/laboratory characteristics established by this analysis will be used to derive a set of pediatric criteria for children who are likely to benefit from an implantable cardioverter defibrillator (ICD). In the future, another multicenter validation study will implement these criteria and, if successful, will demonstrate a significant decrease in the number of SDs in children with HCM.
REC name
East of Scotland Research Ethics Service REC 1
REC reference
15/ES/0129
Date of REC Opinion
7 Aug 2015
REC opinion
Favourable Opinion