Anatomical-MRI Correlations in Adults and Children with Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare disease in which the right ventricular myocardium is replaced by islands of fibro-adipose tissue. Therefore, ventricular re-entry circuits can occur, predisposing the patient to ventricular tachyarrhythmias, as well as dilation of the right ventricle that eventually leads to heart failure. Although it is a rare disease with low prevalence in Europe and the United States, many patients are addressed disproportionately for cardiac magnetic resonance imaging (MRI). The most severe consequence of this condition is sudden cardiac death at a young age due to untreated cardiac arrhythmias. The purpose of this paper is to revise the magnetic resonance characteristics of ARVC, including the segmental contraction abnormalities, fatty tissue replacement, decrease of the ejection fraction, and the global RV dilation. Herein, we also present several recent improvements of the 2010 Task Force criteria that are not included within the ARVC diagnosis guidelines. In our opinion, these features will be considered in a future Task Force Consensus.

Recurrent Syncope in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy

“…The incidence of ARVC is unknown, but there is an estimated prevalence from 1 in 2000 to 1 in 5000 in the general population [ 2 ]. Initial descriptions of ARVC focused on the arrhythmic substrate of some areas of the right ventricle (RV), specifically the subtricuspid RV wall, the RV apex, and the RV outflow tract [ 3 ] with recent studies supporting the presence of a new biventricular triangle, adding the left ventricular (LV) posterolateral wall as part of the pathogenesis [ 4 ]. Currently, efforts have been made to improve our understanding, proposing new measures for early detection and genetic testing in high-risk patients.…”