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Evolving Diagnostic Criteria for Arrhythmogenic Cardiomyopathy

Domenico Corrado1, Alessandro Zorzi1, Alberto Cipriani1

  • 1Department of Cardiac, Thoracic and Vascular Sciences and Public Health University of Padova Italy.

Journal of the American Heart Association
|September 17, 2021
PubMed

Insights

The 2020 International criteria for arrhythmogenic cardiomyopathy (ACM) enhance diagnosis by including left ventricular involvement and cardiac magnetic resonance imaging. These updated guidelines improve detection of the full spectrum of ACM phenotypes.

Area of Science:

  • Cardiology
  • Medical Diagnostics
  • Cardiac Imaging

Background:

  • Previous arrhythmogenic cardiomyopathy (ACM) diagnostic criteria (1994, 2010) accurately identified right ventricular disease but missed left-sided variants.
  • These earlier criteria did not incorporate cardiac magnetic resonance (CMR) late-gadolinium enhancement (LGE) findings, crucial for fibrosis detection.

Purpose of the Study:

  • To review the evolution of ACM diagnostic criteria.
  • To detail the advancements introduced by the 2020 International criteria (Padua criteria).
  • To highlight the improved diagnostic capabilities for the expanding ACM phenotypic spectrum.

Main Methods:

  • Review of historical (1994, 2010) and current (2020) International Task Force criteria for ACM diagnosis.
  • Emphasis on the role of cardiac magnetic resonance (CMR) with late-gadolinium enhancement (LGE) in the 2020 criteria.
  • Inclusion of new criteria for left ventricular (LV) abnormalities and arrhythmias.

Main Results:

  • The 2020 criteria significantly improve the diagnosis of arrhythmogenic biventricular and left ventricular cardiomyopathy.
  • CMR with LGE is now mandatory for characterizing ACM phenotypes and excluding other conditions.
  • New ECG and arrhythmia criteria enhance the identification of left-sided ACM variants.

Conclusions:

  • The 2020 International criteria represent a major advancement in diagnosing the full spectrum of arrhythmogenic cardiomyopathy.
  • Cardiac magnetic resonance imaging is indispensable for accurate ACM diagnosis under the new guidelines.
  • These criteria enhance the ability to detect and characterize both right and left ventricular involvement in ACM.

Keywords:
cardiac magnetic resonancecardiomyopathydiagnosissudden deathventricular arrhythmia

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