Abstract
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a rare inherited cardiomyopathy currently diagnosed using established Task Force criteria consisting of ECHO, MRI, signal averaged ECG (SAECG), Holter monitoring, family history, and histologic tissue data. In early stages of disease, diagnosis is difficult, and these criteria may be insensitive. Anti-DSG2 is an antibody against a cadherin protein (DSG2) required in the formation of desmosomes which connect cardiac myocytes. It has been hypothesized that these antibodies arise from the exposure of novel epitopes to the immune system in the setting of mutations that disrupt the desmosome.
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