Thelansis

  Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a hereditary cardiac disorder characterized by impaired function of the right ventricle (RV) and the occurrence of ventricular arrhythmias, which can ultimately lead to sudden cardiac death. The condition is characterized by the replacement of cardiomyocytes with fibrofatty tissue. ARVD/C is typically inherited in an autosomal dominant manner, but it exhibits incomplete penetrance, and the expression of the disease varies significantly with age. In rare cases, autosomal recessive inheritance can occur with or without cutaneous involvement. The progressive fibrotic replacement process leads to electrical instability, increasing the risk of syncope and sudden cardiac death, or causing alterations in cardiac function, resembling dilated cardiomyopathy, which may result in right- or bi-ventricular heart failure. The diagnosis of ARVD/C is challenging and relies on applying the 2010 Task Force criteria, which consid...