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Arrhythmogenic Cardiomyopathy in the Pediatric Patient

Elizabeth DeWitt1, Dominic Abrams2

  • 1Department of Pediatrics, Harvard Medical School; Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.

Cardiac Electrophysiology Clinics
|November 8, 2025
PubMed

Insights

Arrhythmogenic cardiomyopathy in children is an inherited heart condition causing arrhythmias. A genotype-first approach aids diagnosis and management for better outcomes.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Arrhythmogenic cardiomyopathy (ACM) is an inherited myocardial disorder.
  • It is increasingly diagnosed in pediatric patients, presenting with arrhythmias before structural changes.
  • ACM can affect one or both ventricles.

Purpose of the Study:

  • To emphasize a genotype-first diagnostic approach for pediatric arrhythmogenic cardiomyopathy.
  • To detail clinical features, diagnostic tools, and management strategies for common genetic subtypes.
  • To highlight the importance of early recognition and individualized care.

Main Methods:

  • Review of clinical features, diagnostic tools, and management strategies for pediatric ACM.
  • Focus on genotype-first diagnosis for common genetic subtypes (e.g., PKP2, desmoplakin).
  • Comparison with pediatric conditions like myocarditis and dilated cardiomyopathy.

Main Results:

  • Pediatric ACM presentations can mimic other cardiac conditions.
  • A genotype-first approach is crucial for accurate diagnosis.
  • Management involves antiarrhythmic drugs, catheter ablation, and ICDs.

Conclusions:

  • Early identification of pediatric arrhythmogenic cardiomyopathy is vital.
  • Individualized care plans improve patient outcomes.
  • Future advanced and gene-targeted therapies are anticipated.

Keywords:
Arrhythmogenic cardiomyopathyDesmosomal genesGenotype-firstPediatricVentricular arrhythmia

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