Novel view of arrhythmogenic cardiomyopathy as a “multicellular disease” affecting cardiac sympathetic innervation

Abstract

Arrhythmogenic Cardiomyopathy (AC) is an incurable familial cardiac disease, mainly due to mutations in desmosomal genes, accounting for most cases of stress-related arrhythmic sudden death, in the young and athletes. AC hearts display cardiomyocyte (CM) death and diffuse fibro-fatty lesions, which compromise cardiac contractile performance and generate the substrate for arrhythmias. Correlation between physical/emotional stresses and arrhythmias incriminates sympathetic neurons (SNs), which may be the sought-after killers in disguise.