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Arrhythmogenic right ventricular cardiomyopathy

Alessio Gasperetti1, Cynthia A James1, Firat Duru2

  • 1Division of Cardiology, Department of Medicine, Johns Hopkins University, 600 N. Wolfe St. Blalock 545, Baltimore, MD 21784, USA.

European Heart Journal
|June 5, 2026
PubMed

Insights

Arrhythmogenic cardiomyopathy (ACM) is an inherited heart condition causing sudden cardiac death risk. This review details its shift from phenotype-based to genotype-based diagnosis and management strategies.

Area of Science:

  • Cardiology
  • Genetics
  • Inherited Diseases

Background:

  • Arrhythmogenic cardiomyopathy (ACM) is an inherited heart condition.
  • Characterized by ventricular scarring and fibro-fatty replacement.
  • Associated with significant risk of sudden cardiac death, particularly in young individuals and athletes.

Purpose of the Study:

  • To summarize evolving understanding of arrhythmogenic cardiomyopathy.
  • Highlight the transition in diagnostic and management frameworks.
  • Emphasize the shift from phenotype-first to genotype-first approaches.

Main Methods:

  • Literature review of arrhythmogenic cardiomyopathy research.
  • Analysis of diagnostic and management guideline changes.
  • Focus on the impact of genetic insights.

Main Results:

  • The understanding of ACM has significantly evolved.
  • A paradigm shift towards genotype-first diagnosis is occurring.
  • Genetic testing is increasingly central to ACM management.

Conclusions:

  • The genotype-first approach offers a more precise framework for ACM diagnosis and management.
  • This shift aids in identifying at-risk individuals and guiding therapeutic strategies.
  • Further research is needed to refine genotype-guided interventions for ACM.

Keywords:
Arrhythmogenic cardiomyopathyArrhythmogenic right ventricular cardiomyopathyDesmosomalSudden cardiac death

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