Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Definition: A genetic cardiomyopathy caused by fibrofatty replacement of the right ventricular myocardium, leading to RV dilation, regional akinesia/dyskinesia, ventricular arrhythmias, and risk of sudden cardiac death. Genetics: • Mostly desmosomal mutations — PKP2 (most common), DSG2, DSC2, DSP, JUP • Autosomal dominant with variable penetrance. • Sometimes associated with exercise-induced progression. --- Pathology Loss of desmosomal adhe
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Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Definition: A genetic cardiomyopathy caused by fibrofatty replacement of the right ventricular myocardium, leading to RV dilation, regional akinesia/dyskinesia, ventricular arrhythmias, and risk of sudden cardiac death. Genetics: • Mostly desmosomal mutations — PKP2 (most common), DSG2, DSC2, DSP, JUP • Autosomal dominant with variable penetrance. • Sometimes associated with exercise-induced progression. --- Pathology Loss of desmosomal adhe
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