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The Many Faces of Arrhythmogenic Cardiomyopathy: An Overview

Hanna J Tadros1, Christina Y Miyake1,2, Debra L Kearney3

  • 1Department of Pediatrics, Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.

The Application of Clinical Genetics
|November 7, 2023
PubMed

Insights

Arrhythmogenic cardiomyopathy (AC) is a heart muscle disease affecting cardiomyocytes, leading to arrhythmias. This review details AC

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Arrhythmogenic cardiomyopathy (AC) is characterized by electromechanical uncoupling of cardiomyocytes.
  • Histologic changes in AC lead to arrhythmogenic clinical features.
  • AC can affect the right ventricle, both ventricles, or the left ventricle alone.

Purpose of the Study:

  • To review the epidemiology, etiologies, presentations, evaluation, and management of arrhythmogenic cardiomyopathy.
  • To detail AC across the age continuum.
  • To discuss evolving diagnostic criteria and genotype-phenotype correlations.

Main Methods:

  • Literature review of recent data on arrhythmogenic cardiomyopathy.
  • Synthesis of information on AC epidemiology, etiology, and clinical presentation.
  • Analysis of diagnostic modalities, criteria, and management strategies.

Main Results:

  • AC understanding has evolved, including its variable ventricular involvement.
  • Diagnostic criteria and modalities for AC have continuously improved.
  • Knowledge of AC clinical features across age groups and genotype-phenotype links has advanced.

Conclusions:

  • Arrhythmogenic cardiomyopathy is a complex disease with evolving diagnostic and management paradigms.
  • Comprehensive understanding across the age spectrum is crucial for effective patient care.
  • Continued research into genotype-phenotype correlations will refine AC diagnosis and treatment.

Keywords:
arrhythmiasarrhythmogenic cardiomyopathycardiomyopathygenotype-to-phenotype correlationpediatrics

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