Editorial
A novel desmin mutation causing severe left ventricular arrhythmogenic cardiomyopathy/dysplasia
Abstract
Arrhythmogenic cardiomyopathy/dysplasia (AC) is a hereditary disorder characterized by degeneration of cardiomyocytes and their subsequent replacement by fat and fibrous tissue mainly, but not exclusively, in the right ventricle (RV) (1-3). Such changes lead to conduction abnormalities that provide the substrate for arrhythmogenesis (4,5).