Keywords
Arrhythmogenic right ventricular cardiomyopathy; Diagnosis; Prognosis; Treatment
Abstract
Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC) is a genetic form of cardiomyopathy affecting primarily the Right Ventricle (RV) but also may involves the Left Ventricle (LV) and may culminate in life-threatening ventricular arrhythmias, Sudden Cardiac Death (SCD) and/or heart failure. In most cases it is transmitted with an autosomal dominant pattern of inheritance, with incomplete penetrance and variable expression, but there are also some rare autosomal recessive forms as Naxos disease and Carvajal syndrome.
The term “cardiomyopathy” should be preferred to “dysplasia”, as already suggested by WHO/ International Society and Federation of Cardiology in 1996 and Maron, about classification of cardiomyopathy in 2006 [1,2].
Citation
Dendramis G. Arrhythmogenic Right Ventricular Cardiomyopathy: From Diagnosis to Treatment. SM Vasc Med. 2016; 1(1): 1005.