A case of arrhythmogenic right ventricular cardiomyopathy presenting with progressive right ventricular failure and recurrent multifocal monomorphic ventricular tachycardia during 15 years of follow-up

摘要

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a multigenic form of cardiomyopathy characterized by myocardial loss and fibrofatty replacement mainly in the right ventricle. Progressive right ventricular dysfunction, ventricular arrhythmias, and sudden cardiac death are the clinical picture of this disease. Despite its clinical importance as a cause of sudden death, ARVC is likely to be under-recognized. In case reports about ARVC, disease characteristics such as arrhythmias, images, and genes are described in fragments. Little is reported about the long-term course of ARVC in the same patient. In this report, we present a case of a 68-year-old male who was diagnosed with ARVC after his first episode of ventricular tachycardia. Both mechanical and electrical progression were seen during the 15 years of follow-up, requiring the modification of disease management. This report could help improve the understanding of this rare disease, and the way of its management.<>Learning objective: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a multigenic form of cardiomyopathy characterized by fibrofatty degeneration mostly in right ventricular myocardium. Despite its importance as a cause of sudden death, ARVC is likely to be under-recognized. This paper describes the progressive course of ARVC confirmed both clinically and pathologically during 15 years of follow-up, which could help improve understanding of this disease.>