Multimodality Imaging in Arrhythmogenic Left Ventricular Cardiomyopathy

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.

Keywords: arrhythmogenic cardiomyopathy; cardiac magnetic resonance; echocardiography; multimodality imaging; positron emission tomography.

Figure 1

Cardiac magnetic resonance images showing myocardial fibrosis (as late gadolinium enhancement areas) limited to the right ventricle (black arrows; A), also extended to the left ventricle (white arrows; B), or with dominant left ventricular involvement («ring-like» late gadolinium enhancement pattern; C).

Figure 2

The role of 18-FDG positron emission tomography in arrhythmogenic cardiomyopathy. Abbreviations: EMB, endomyocardial biopsy.