Beyond Arrhythmogenic Right Ventricular Cardiomyopathy: A Scoping Review of Arrhythmogenic Cardiomyopathy Variants

Abstract

BACKGROUND Arrhythmogenic cardiomyopathy (ACM) is a rare heart muscle disorder consisting of three variants: arrhythmogenic right ventricular cardiomyopathy (ARVC), arrhythmogenic left ventricular cardiomyopathy (ALVC), and arrhythmogenic biventricular cardiomyopathy (ABVC). This condition is a significant cause of sudden cardiac death (SCD), especially among young athletes.
Objectives: Due to the lack of research on the clinical outcomes associated with each ACM variant, this study aims to explore the entire phenotypic spectrum of ACM through a comparative lens and to explore its recent reconceptualization as a biventricular condition rather than primarily right-sided cardiomyopathy.

METHODS Using a combination of keywords, a database search was performed on PubMed, Cochrane Central Register of Controlled Trials (CENTRAL), Science Direct and Google Scholar databases. Through thorough discussion, authors collectively extracted, elaborated, and assessed their findings to enhance reliability.

RESULTS Our findings suggested that ABVC may be the most prevalent and least favorable form of ACM, followed by ALVC and ARVC. While right ventricular involvement in ACM has been linked to a higher arrhythmic burden, left ventricular involvement was associated with higher risks of heart failure, cardiac transplantation, and Hot phases. The recently proposed European Task Force Criteria report can potentially rectify previous underdiagnosis and diagnostic delays associated with ALVC and ABVC. This study also highlights the paucity of clinical trials on ACM.

CONCLUSION Distinct outcomes have been associated with each variant, suggesting that tailored management for each variant of ACM may be required. Further research into the clinical outcomes and management of each variant of ACM is essential to improve patient care.