Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Type 1: A Light on Molecular Mechanisms

Genetics of arrhythmogenic right ventricular cardiomyopathy.

Recent advances in molecular genetics of arrhythmogenic right ventricular cardiomyopathy (ARVD) are reviewed. In particular, the finding of mutations in the gene coding for cardiac ryanodine receptor (hRYR2), both in patients affected with ARVD2 and in patients affected with catecholaminergic ventricular arrhythmias or with familial ventricular tachyarrhythmia, is discussed. Novel data support ...

Assessing the significance of pathogenic mutations and autopsy findings in the light of 2010 arrhythmogenic right ventricular cardiomyopathy diagnostic criteria: a clinical challenge.

MRI of arrhythmogenic right ventricular cardiomyopathy/dysplasia.

Magnetic Resonance Imaging (MRI) is currently considered as the noninvasive modality of choice for evaluation of patients with suspected Arrhythmogenic Right Ventricular Dysplasia (i.e., right ventricular dysplasia). As arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) it is included in the WHO classification of cardiomyopathies. It has the unique ability to provide tissue char...

Arrhythmogenic Ventricular Dysplasia/Cardiomyopathy: Insights from the Rationale of Disease Nomenclature and Clinical Perspectives

“Arrhythmogenic right ventricular dysplasia” (ARVD), a heart muscle disorder characterized by the presence of fibro-fatty tissue and ventricular electrical vulnerability related to sudden death, was first described in 1977 by a French team. Since then, other terms such as “arrhythmogenic right ventricular cardiomyopathy” (ARVC), “arrhythmogenic cardiomyopathy” (AC), “left-dominant arrhythmogeni...

Science and practice of arrhythmogenic cardiomyopathy: A paradigm shift

INTRODUCTION The clinical, genetic, and molecular paradigm of arrhythmogenic right ventricular cardiomyopathy (ARVC) has markedly progressed through the last three decades, shifting from the classical ARVC as a progressive condition characterized by fibrofatty replacement of the right ventricle, into a wider spectrum of arrhythmogenic cardiomyopathy (AC), which covers ARVC with its various clin...