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Contemporary diagnostic approach to arrhythmogenic cardiomyopathy: The three-step work-up

Francesca Graziano1, Alessandro Zorzi2, Alberto Cipriani2

  • 1Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, Italy; Department of Sports Medicine, Semmelweis University, Budapest, Hungary; Heart and Vascular Center, Semmelweis University, Budapest, Hungary.

Trends in Cardiovascular Medicine
|September 28, 2024
PubMed

Insights

Diagnosing Arrhythmogenic Cardiomyopathy (ACM) has evolved significantly. Recent European Task Force criteria (2023) improve detection by incorporating left ventricular disease and cardiac MRI findings, advancing ACM diagnosis.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Diagnostics

Background:

  • Arrhythmogenic Cardiomyopathy (ACM) is a genetic heart disorder causing myocardial scarring, electrical instability, and potential heart failure.
  • Diagnosing ACM is complex due to the absence of a single definitive test, necessitating a multi-faceted approach.

Purpose of the Study:

  • To review the 20-year evolution of diagnostic criteria for Arrhythmogenic Cardiomyopathy.
  • To highlight advancements in understanding ACM pathobiology and morpho-functional features.

Main Methods:

  • Review of diagnostic criteria from 1994 to 2023.
  • Analysis of the progression from right ventricular focus to comprehensive assessment including left ventricular variants.
  • Inclusion of cardiac magnetic resonance imaging (CMR) tissue characterization in diagnostic frameworks.

Main Results:

  • Initial criteria (1994, 2010) focused on right ventricular abnormalities.
  • The 2020 Padua criteria expanded diagnosis to include left ventricular ACM and CMR findings.
  • The 2023 European Task Force criteria represent a refined, internationally recognized standard for ACM diagnosis.

Conclusions:

  • Diagnostic criteria for ACM have progressively improved over two decades.
  • Modern criteria, like the 2023 European Task Force guidelines, offer enhanced accuracy and broader applicability.
  • These advancements reflect a deeper understanding of ACM's complex pathology.

Keywords:
Arrhythmogenic cardiomyopathyCardiac magnetic resonanceDiagnosisSudden deathVentricular arrhythmia

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