Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a chronic, progressive, heritable cardiomyopathy, characterized by fibrofatty replacement and dysfunction of right ventricular. The first symptoms of ARVC/D include palpitations, effort-induced syncope, dyspnea, chest pain, whereas sudden cardiac death is the first clinical manifestation during physical activity, training or just after physical activity in 10% of cases. It is one of the most common causes of sudden cardiac death in young athletes, accounting for approximately 22% of cases. ARVC/D is an autosomal dominant inherited disease. There are main five disease-causing genes which encode desmosomal proteins and their dysfunction causes myocyte detachment from one another and cell death - plakoglobin, desmoplakin, plakophilin-2, desmoglein and desmocollin-2. The diagnosis of ARVC/D is made using a combination of family history, non-invasive and invasive tests based on Task Force 2010 criteria. Clinical management of ARVC/D patients includes betablockers, antiarrhythmic drugs and drugs for heart failure. In the case of ineffective pharmacological therapy, catheter ablation is a therapeutic option. The main prevention of sudden cardiac death is implantable defibrillator (ICD). The indications to ICD implantation in ARVC/D depend on risk group for major arrhythmic event. Early identification of patients, correct prevention and treatment can prevent sudden cardiac death. Data from prospective studies/registries with larger number of patients and longer follow-up are required to provide evidence-based recommendations for the best care of ARVC/D patients. This article provides a review of a clinical case according to the newest literature about diagnostic criteria, treatment and prevention of the disease.
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