Heart failure & cardiomyopathies
Cardiomyopathies in children and adolescents: aetiology, management, and outcomes in the European Society of Cardiology EURObservational Research Programme Cardiomyopathy and Myocarditis Registry
Authors' conclusion
The findings confirm the heterogeneous aetiology of childhood cardiomyopathies and show a high frequency of familial disease. Outcomes differed by cardiomyopathy subtype
Study context
- Population
- Children and adolescents with cardiomyopathy, ESC EURObservational Cardiomyopathy and Myocarditis Registry (June 2014–December 2016)aged ≤18 years
- Participants
- 633 children
- Exposure
- HCM vs DCM vs RCM vs ARVC
- HCMn = 388
- DCMn = 206
- RCMn = 28
- ARVCn = 11
Methodological detailsmulticentre, 23 referral centres in 14 countries
- Multicentre
- 23 referral centres in 14 countries
Main finding
As reported in the abstract
Baseline characteristics
- Median age at diagnosis (IQR 0-10)
- 4.0 years
- Male
- 372 (58.8%)
- Receiving cardiac medication
- 621 (98.1%)
- ICD
- 80 (12.6%)
- Familial disease
- 253/535, 47.3%
- Genetic testing performed
- 414 (67.8%)
- Pathogenic or likely pathogenic variant among tested
- 250 (60.4%)
- Rare disease phenocopies
- 177 (28.0%)
Observational data: associations, not effects.
Further findings
| Outcome | HCM | DCM | RCM | ARVC | |
|---|---|---|---|---|---|
| Death · median follow-up 12.5 months (IQR 11.3-15.3 months) | 2.6% | 3.0% | 16.0% | Not reported in the abstract | ARVC: not reported in the abstract |
| HF events | Not reported in the abstract | Not reported in the abstract | 36.0% | Not reported in the abstract | HCM, DCM, ARVC: not reported in the abstract |
- Death — Overall deaths
- 18 (3.3%)
Rare disease phenocopies, age <10 vs ≥10 years
- Under 10 years vs older
- 142 (30.9%) vs. 35 (19.6%); P = .003
HF events
- Most frequent in RCM
Original publication
Cardiomyopathies in children and adolescents: aetiology, management, and outcomes in the European Society of Cardiology EURObservational Research Programme Cardiomyopathy and Myocarditis Registry. Eur Heart J. Published 1 March 2024. doi:10.
Summarised from the published abstract; every number is checked against it