Arrhythmogenic right ventricular dysplasia.

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a genetic disorder that is characterized by ventricular arrhythmias and structural abnormalities of the right ventricle. Due to significant heterogeneity in its manifestation, the diagnosis of ARVD is challenging and requires a multifaceted approach to patient evaluation. It is important to not rush and diagnose ARVD prematurely, as the implications both for the patient and also for family members are enormous. Similarly, it is important for clinicians to be aware of this condition because it is potentially life threatening. There are three keys aspects to treatment once a diagnosis is established. The first issue concerns risk stratification and deciding whether to implant an implantable cardioverter defibrillator (ICD). We currently advise ICD implantation for probands who meet the full criteria for the disease, especially if they have experienced cardiac syncope, sustained ventricular tachycardia, or have severe right ventricular or left ventricular dysfunction. In addition, we feel there are sufficient observational clinical data and scientific data from animal models to advise that both competitive sports and high-level athletics be prohibited. We advise our patients to generally limit their activity to activities such as walking and golf. Finally, it is our opinion that most patients with ARVD should be treated with both a β-blocker as well as an angiotensin-converting enzyme inhibitor, provided these drugs are well tolerated.