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Proposed diagnostic criteria for arrhythmogenic cardiomyopathy: European Task Force consensus report

Domenico Corrado1, Aris Anastasakis2, Cristina Basso1

  • 1Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padua Medical School, Italy.

International Journal of Cardiology
|October 16, 2023
PubMed

Insights

New diagnostic criteria improve the identification of arrhythmogenic cardiomyopathy (ACM), a heart muscle disease involving myocardial scarring. These updated guidelines enhance the detection of both right and left-sided variants for better patient management.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Diagnostics

Background:

  • Arrhythmogenic cardiomyopathy (ACM) is a myocardial disease defined by "non-ischemic" scarring, leading to electrical instability.
  • Previous diagnostic criteria (1994, 2010) accurately diagnosed arrhythmogenic right ventricular cardiomyopathy (ARVC) but lacked sensitivity for left-sided variants (ALVC, ABVC).
  • Under-recognition of ACM, particularly left-sided forms, has persisted due to the absence of specific diagnostic criteria.

Purpose of the Study:

  • To refine and upgrade existing diagnostic criteria for arrhythmogenic cardiomyopathy (ACM).
  • To incorporate advanced imaging techniques, specifically cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE), for comprehensive disease characterization.
  • To establish internationally recognized criteria for improved diagnosis and management of all ACM phenotypes.

Main Methods:

  • Development of refined diagnostic criteria by a European expert Task Force, building upon the 2020 Padua criteria.
  • Integration of cardiac magnetic resonance (CMR) findings, including the "ring-like" pattern of LGE/scar, for characterizing myocardial tissue.
  • Inclusion of electrocardiogram (ECG) abnormalities related to left ventricular (LV) depolarization/repolarization and LV-originating arrhythmias.

Main Results:

  • The proposed criteria enhance the diagnosis of ACM across its spectrum, including right, biventricular, and left variants.
  • Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) is crucial for identifying myocardial scar, with a "ring-like" pattern being a hallmark of ALVC.
  • New criteria address LV depolarization/repolarization abnormalities and ventricular arrhythmias, improving diagnostic accuracy.

Conclusions:

  • The upgraded diagnostic criteria provide a robust framework for identifying and managing arrhythmogenic cardiomyopathy (ACM).
  • Enhanced diagnostic sensitivity, particularly for left-sided ACM variants, is achieved through the integration of CMR and ECG findings.
  • These criteria aim to improve clinical recognition and patient outcomes for individuals with ACM.

Keywords:
Cardiac magnetic resonanceCardiomyopathyDiagnosisSudden deathVentricular arrhythmia

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