Related Experiment Video
Updated: Jan 11, 2026

Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia
Published on: December 22, 2023
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.
None:
Arrhythmogenic cardiomyopathy is an inherited myocardial disorder increasingly recognized in pediatric patients. It is characterized by arrhythmias that often precede structural abnormalities and can involve the right, left, or both ventricles. This article emphasizes a genotype-first diagnostic approach, detailing the clinical features, diagnostic tools, and management strategies for common genetic subtypes including PKP2, desmoplakin, and others. Pediatric presentations may mimic myocarditis or dilated cardiomyopathy, necessitating thorough evaluation. Management includes antiarrhythmic medications, catheter ablation, and implantable cardioverter defibrillators, with advanced therapies and gene-targeted treatments on the horizon. Early recognition and individualized care are critical for improving outcomes in affected children.
Related Concept Videos
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy IV: Restrictive Cardiomyopathy
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Mechanism of Cardiac Arrhythmias
Disturbances in Heart Rhythm
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias

