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Multimodality Imaging in Arrhythmogenic Left Ventricular Cardiomyopathy
Emanuele Monda1,2, Marta Rubino1, Giuseppe Palmiero1
1Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.
Insights
Arrhythmogenic cardiomyopathy (ACM) involves heart muscle disease with fibrosis, leading to arrhythmias. This review focuses on arrhythmogenic left ventricular cardiomyopathy (ALVC) and its diagnosis using multimodality imaging.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Arrhythmogenic cardiomyopathy (ACM) is a spectrum of myocardial diseases characterized by fibrotic or fibrofatty replacement.
- Arrhythmogenic left ventricular cardiomyopathy (ALVC) specifically affects the left ventricle, causing ventricular arrhythmias.
- Diagnostic criteria for ALVC exist but require genetic testing for confirmation due to overlapping features with other cardiac conditions.
Purpose of the Study:
- To review the role of multimodality imaging in diagnosing arrhythmogenic left ventricular cardiomyopathy (ALVC).
- To highlight the importance of various imaging techniques in ALVC diagnosis, risk stratification, and management.
Main Methods:
- Review of current literature on arrhythmogenic left ventricular cardiomyopathy (ALVC).
- Discussion of multimodality imaging techniques including echocardiography, cardiac magnetic resonance, and nuclear imaging.
- Emphasis on the integration of genetic testing for diagnostic confirmation.
Main Results:
- Multimodality imaging provides crucial data for ALVC diagnosis and differential diagnosis.
- Imaging aids in risk stratification for sudden cardiac death in ALVC patients.
- Genetic testing is essential for confirming ALVC diagnosis, especially when imaging findings overlap with other cardiomyopathies.
Conclusions:
- Multimodality imaging is indispensable for the comprehensive evaluation of patients with arrhythmogenic left ventricular cardiomyopathy (ALVC).
- The integration of advanced imaging with genetic analysis improves diagnostic accuracy and patient management for ALVC.
- Further research into optimizing imaging protocols and understanding disease mechanisms in ALVC is warranted.
Abstract:
The term arrhythmogenic cardiomyopathy (ACM) describes a large spectrum of myocardial diseases characterized by progressive fibrotic or fibrofatty replacement, which gives the substrate for the occurrence of ventricular tachyarrhythmias and the development of ventricular dysfunction. This condition may exclusively affect the left ventricle, leading to the introduction of the term arrhythmogenic left ventricular cardiomyopathy (ALVC). The clinical features of ALVC are progressive fibrotic replacement with the absence or mild dilation of the LV and the occurrence of ventricular arrhythmias within the left ventricle. In 2019, the diagnostic criteria for the diagnosis of ALVC, based on family history and clinical, electrocardiographic, and imaging features, have been proposed. However, since the significant clinical and imaging overlap with other cardiac diseases, genetic testing with the demonstration of a pathogenic variant in an ACM-related gene is required for diagnostic confirmation. In ALVC, the multimodality imaging approach comprises different imaging techniques, such as echocardiography, cardiac magnetic resonance, and cardiac nuclear imaging. It provides essential information for the diagnosis, differential diagnosis, sudden cardiac death risk stratification, and management purposes. This review aims to elucidate the current role of the different multimodality imaging techniques in patients with ALVC.
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