Complete Atrioventricular Block as a First Presentation of Arrhythmogenic Right Ventricular Cardiomyopathy in a Middle-Aged Male: A Nouvel Clinical Entity

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is arare inherited cardiac disease that presents with progressive right ventricular (RV) dysfunction due to fibro-fatty replacement of the myocardium and associated with an elevated risk of ventricular arrhythmias and sudden cardiac death. ARVC/D has a predominantly autosomal dominant inheritance, although recessive forms associated with a cutaneous phenotype, such as Naxos disease and Carvajal syndrome, are also observed. The golden standard for diagnosing ARVC/D is an endomyocardial biopsy demonstrating fibro-fatty replacement. Our case concerns a middle-aged male patient with rare bradyarrhythmia (complete atrioventricular block) associated with signs and symptoms of right heart failure who was diagnosed with arrhythmogenic right ventricular cardiomyopathy ARVC died shortly upon admission at the emergency department.