ABSTRACT
Arrhythmogenic right ventricular cardiomyopathy is a genetically determined cardiomyopathy clinically characterized by life-threatening ventricular arrhythmias and/or heart failure predominantly involving the right ventricle. The prevalence of the disease among the general population has been estimated to range from 1:1000 to 1:5000 with similar distribution in Europe and North America. Arrhythmogenic right ventricular cardiomyopathy is a genetically determined cardiomyopathy with heterogeneous inheritance and clinical phenotype with variable penetrance and clinical severity of the disease among family trees. Multiple genes and several exogenous factors interacting with the genetic substrate are probably involved in the pathologic process leading to the clinical expression of the disease. Arrhythmogenic right ventricular cardiomyopathy has a wide spectrum of clinical manifestations ranging from palpitations, syncope, and sudden cardiac death especially in young males, to congestive heart failure. Due to the heterogeneous nature of the disease and its clinical manifestations, there is no single criteria allowing a definite diagnosis.
