Varied Presentation of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C): A Case Series

Arrhythmogenic right ventricular dysplasia (ARVD) is a genetically predisposed form of cardiomyopathy that mainly affects young individuals resulting in fatal ventricular arrhythmias leading to sudden cardiac death. ARVD has 50% of cases that involve both the right ventricle (RV) and left ventricle (LV), but only a small number of cases involve an isolated left ventricle. In this case series, five patients (four males and one female) with a diagnosis of ARVD presented to our center with varied clinical presentations across a wide range of age groups. The MRI of all five cases showed dilated right atrium (RA)/RV with right ventricular free wall dyskinesia. Two-dimensional (2D) MRI showed aneurysmal outpouching with diffuse free wall enhancement. Automated implantable cardioverter defibrillator (AICD) was implanted uneventfully in all five patients, and the patients were discharged with oral medications such as low-dose diuretics, beta-blockers, spironolactone, angiotensin-converting enzymes (ACE) inhibitors, amiodarone, and anxiolytics. Until now, the patients were doing well on follow-up visits. The therapeutic management of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) has evolved over the years and continues to be an important challenge. To further improve risk stratification and treatment of patients, more information is needed on natural history, long-term prognosis, and risk assessment. Special attention should be focused on the identification of patients who would benefit from implantable cardioverter-defibrillator (ICD) implantation in comparison to pharmacological and other nonpharmacological approaches.