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
Summary
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.
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