Reduced Desmoplakin immunofluorescence signal in arrhythmogenic cardiomyopathy with epicardial right ventricular outflow tract tachycardia

Arrhythmogenic cardiomyopathy (ACM), formerly known as arrhythmogenic right ventricular dysplasia, is a primary myocardial disorder characterized by ventricular arrhythmias and sudden cardiac death (SCD).1 Myocardial desmosomes are cell–cell junctions that reside within the intercalated disc. They consist of members of the cadherin family (desmocollin-2, desmoglein-2), which span the membrane mechanically coupling adjacent cells, and members of the plakin and armadillo families: plakoglobin (PKG), plakophilin-2 (PKP2), and desmoplakin (DSP1), which connect the cadherin complexes to the intermediate cytoskeleton filaments.