36. What is arrhythmogenic right ventricular dysplasia?

Arrhythmogenic right ventricular dysplasia (ARVD) or arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic condition (autosomal dominant) affecting the desmosomes of the cardiac myocyte. This results in fatty replacement of the right ventricular myocardium.

As the name implies, the predominant feature of ARVD is arrhythmia, usually in the form of ventricular tachycardia. This ventricular tachycardia is frequently from the right ventricular outflow tract also known as adenosine sensitive ventricular tachycardia. Premature ventricular contractions are common. Sudden cardiac death from ventricular fibrillation is a primary concern.