To investigate the incidence, aetiology, and outcomes of pediatric cardiomyopathy in a national cohort in Northern Ireland.
Approach:
Study Design: Retrospective cohort study using chart review and local pediatric cardiology database.
Population: Children diagnosed with phenotype-positive pediatric cardiomyopathy managed at the regional Pediatric Cardiology Centre from 2003 to 2025.
Data Collection: Demographic, clinical, genetic characteristics, and clinical outcomes were collected.
Statistical Analysis: Incidence rates calculated per 100,000 children; temporal trends assessed using generalized linear models.
Key Findings:
Eighty-four children diagnosed with pediatric cardiomyopathy; 24 with DCM, 38 with HCM, 2 with RCM, 1 with arrhythmogenic cardiomyopathy, 12 with left ventricular non-compaction as a morphological feature, 3 with non-dilated left ventricular cardiomyopathy, and 4 with unspecified cardiomyopathy.
Median age at diagnosis was 2 years; DCM patients were diagnosed at a median age of 0.28 years.
61% of patients had a pathogenic or likely pathogenic genetic variant identified.
Interpretation:
The study provides insights into the incidence and genetic features of pediatric cardiomyopathy in Northern Ireland.
Limitations:
Retrospective design may introduce bias.
Limited generalizability due to single-center study.
Conclusion:
The study highlights the incidence and genetic landscape of pediatric cardiomyopathy.
by Scott Kendall, Shannon Scott, Peter McClung, Joy McCance, Nawab Ali, Gillian Rea, William Wright, Jane Murray, Alison Muir, Martin Dempster, Terrence Prendiville, Pascal McKeown, Frank Casey
