Right ventricular dysplasia: management and treatment in light of current evidence

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare cardiovascular disease that predisposes to ventricular arrhythmias potentially leading to sudden cardiac death (SCD). ARVC varies considerably with multiple clinical presentations, ranging from no symptoms to cardiac arrhythmias to SCD. ARVC prevalence is not well known, but the estimated prevalence in the general population is 1:5000. Diagnosis of ARVC can be made by using the Revised European Society of Cardiology criteria for ARVC that includes ventricular structural and functional changes, ECG abnormalities, arrhythmias, family and genetic factors. The management of ARVC is focused on prevention of lethal events such as SCD. Implantable cardioverter defibrillator placement is the only proven mortality benefit in treatment of ARVC. Other treatment strategies include medications such as beta blockers and antiarrhythmics, radiofrequency ablation, surgery, cardiac transplantation, and lifestyle changes. All these interventions help in symptomatic treatment but none of them have proved to decrease mortality rates. ARVC is a progressive disease that leads to SCD if not treated appropriately. Management of these diseases has been a challenge for physicians. With the advent of technology and many new drugs/devices under clinical investigation, this might change in the future. However, while advances in technologies have helped elucidate many aspects of these diseases, many mysteries still remain of this unique disease. With continued research, we can expect more cost-effective and patient-friendly drug therapies and ablation techniques to be developed in the near future.