Arrhythmogenic Right Ventricular Cardiomyopathy Presenting With VT Electrical Storm

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a right ventricular disease caused by desmosomal gene mutations leading to fibro-fatty replacement of the myocardium causing ventricular arrhythmias such as ventricular tachycardia (VT). A 59-year-old female presented with new onset VT manifesting as shortness of breath and chest discomfort. Diagnostic workup revealed right ventricular dilation/dysfunction on echocardiogram, VT with left bundle branch block (LBBB) and diffuse T wave inversions (TWIs) on EKG. She was discharged with a diagnosis of ARVC. Later, she developed VT storm, treated with epi-endocardial ablation for complete resolution of recurrent VT. Our case demonstrates the rare presentation of ARVC as ventricular storm and the various management strategies such as anti-arrhythmics, automated implantable cardioverter-defibrillator (AICD) and ablation procedures.