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.

Arrhythmogenic Left Ventricular Cardiomyopathy: From Diagnosis to Risk Management

“…Additionally, a group of ALVC patients with FDG-PET uptake have also reported sVT, providing further evidence for the involvement of myocardial inflammation in the development of the disease [43].…”

A case report of isolated arrhythmogenic left ventricular cardiomyopathy: phenotypes, diagnosis, and treatment

“…Multimodality imaging, such as echocardiography, CMRI, and black blood imaging, is imperative in the timely assessment of ventricular aneurysm morphology, function, and tissue characterization. 7 , 8 About 50% of patients with ACM had mutations in genes encoding cardiac desmosomal proteins, showing that gene variants, including DSP, RYR2, TTN, PKP2, DSC2, and DSG2, were closely related to ACM. 9 , 10 DSP is the main component of desmosomes that interacts with PKP to link DSC and DSG to intermediate filaments.…”

Personalized Management of Sudden Death Risk in Primary Cardiomyopathies: From Clinical Evaluation and Multimodality Imaging to Ablation and Cardioverter-Defibrillator Implant

“…According to the 2020 international criteria, the diagnosis of ACM is focused on a multiparametric approach and needs at least one morpho-functional or structural criterion plus a pathogenic mutation [ 30 ]. Among these proposed diagnostic criteria, two are the main findings detectable by echocardiography: firstly, the presence of global LV systolic dysfunction, represented by depression of LV ejection fraction or reduction in LV global longitudinal strain, with or without LV dilatation; secondly, the detection of regional LV hypokinesia or akinesia of the free wall, septum, or both, with a preserved LV global systolic performance.…”