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. Author manuscript; available in PMC: 2017 Apr 1.
Published in final edited form as: Circ Arrhythm Electrophysiol. 2016 Apr;9(4):10.1161/CIRCEP.115.003631 e003631. doi: 10.1161/CIRCEP.115.003631

Figure 6.

Figure 6

Schematic representation of the ARVC/ course from desmosomal-gene mutation to phenotypic expression and complex interaction of arrhythmogenic mechanisms leading to cardiac arrest due to ventricular fibrillation. ARVC is a heart muscle disease caused by genetically defective DS proteins. The typical phenotype is characterized by fibro-fatty myocardial replacement predisposing to scar-related ventricular arrhythmias which may lead to SCD due to ventricular fibrillation. Experimental data support the concept that life-threatening ventricular arrhythmias may occur in the absence of structural changes due to a purely electrical mechanism: the loss of expression of DS-proteins may induce electrical ventricular instability and SCD by a concomitant sodium channel dysfunction with current reduction (similarly to the electrogenesis of ventricular fibrillation in patients with Brugada syndrome), as a consequence of the cross-talk between these molecules at the intercalated discs.