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Understanding Childhood Cardiomyopathies: Insights from the First European Registry

18 Mar 2024
cardiomyopathy

Childhood-onset cardiomyopathies represent a rare and poorly understood subset of cardiac disorders. In a recent study published in the European Heart Journal, investigators delved into the demographics and prognosis of pediatric cardiomyopathy patients within the inaugural European Cardiomyopathy Registry.

Data were prospectively gathered from individuals aged 1 to under 18 years participating in the European Society of Cardiology EURObservational Research Programme Cardiomyopathy and Myocarditis long-term registry spanning from June 2014 to December 2016.

Key Findings

Prevalence and Subtypes: Hypertrophic cardiomyopathy (HCM) was the most common subtype, accounting for 61.3% (n = 388) of cases, followed by dilated cardiomyopathy (DCM) at 32.5% (n = 206), restrictive cardiomyopathy (RCM) at 4.4% (n = 28), and arrhythmogenic right ventricular cardiomyopathy (ARVC) at 1.7% (n = 11).

Demographic Trends: The median age at diagnosis was 4.0 years (interquartile range: 0–10), with a notable male predominance across all subtypes (58.8%, n = 372), except for DCM cases diagnosed before 10 years of age.

Genetic Associations: Genetic testing was conducted in 67.8% (n = 414) of patients, revealing pathogenic or likely pathogenic variants in 60.4% (n = 250) of tested cases. Familial disease was identified in 47.3% (n = 253) of patients.

Outcomes: Over a median follow-up of 12.5 months (IQR: 11.3–15.3), 3.3% (n = 18) of patients died, with heart failure events most common in RCM patients (36.0%).

Insights and Implications

These findings underscore the heterogeneous nature of childhood cardiomyopathies and highlight the importance of tailored evaluation and treatment strategies. Genetic testing plays a crucial role in identifying familial cases and guiding management. Differential outcomes across subtypes emphasize the need for personalized care pathways, informing targeted interventions to optimize prognosis in pediatric cardiomyopathy patients.

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Original article: Kaski J. Eur Heart J. 2024 Mar 1:ehae109.

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