Arrhythmogenic right ventricular cardiomyopathy associated ventricular tachycardia misdiagnosed as idiopathic right ventricular outflow tract origin and its management

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an autosomal dominant genetic form of cardiomyopathy (CM), which primarily affects the right ventricle (RV) and results in life threatening ventricular arrhythmias and sudden cardiac death (SCD). Diagnosis is difficult due to the broad spectrum of phenoltypic variations, especially in the early stage. Clinical suspicion should be raised in the setting of refractory ventricular tachycardia originating from the RV, and the final diagnosis could be made based on the combination of electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Implantable cardioverter-defibrillator (ICD) implantation is an effective option for the treatment of ARVC.