Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) in Adults: Case Report with Review of the Literature

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a cardiomyopathy of genetic origin, caused by abnormalities of desmosomes, characterized on the physiopathological level by a fibro-adipose infiltration replacing the myocardium of the right ventricle and the clinical level by an electrical instability leading to ventricular arrhythmias. ARVC/D peaks in frequency between the ages of 30 and 50. Diagnostic criteria have been established to retain the diagnosis of ARVC/D. Imaging and especially magnetic resonance imaging (MRI) play an important role in this diagnosis. We report the observation of a 48-year-old man, a former smoker, with a family history of the sudden death of a sister during a bicycle race, and who has been complaining for several years of palpitations. Clinical presentation, electrical signs, cardiac ultrasound and imaging findings lead to the diagnosis of ARVC/D. According to this observation, the authors describe the authors review the literature of this rare entity and discuss the different therapeutic approaches.