Atypical presentation of arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is a genetic-based disorder, autosomal dominant transmission, in which normal myocardium is replaced by adipose or fibroadipose tissue. It is thus one of the main causes of sudden cardiac death (SCD) in young people. Sustained ventricular tachycardia (SVT) or nonsustained ventricular tachycardia (NSVT) are common symptoms of this disorder. Heart failure (HF) is a less frequent symptom. This is a clinical case of a 53-year-old patient presenting dyspnea and signs of HF in a context of supraventricular arrhythmia. Echocardiogram showed dilation of right cavities. This was the first approximation towards a diagnosis of this peculiar manifestation of ARVC.

Keywords: Heart failure; Cardiomyopathy; Sudden death; Arrhythmogenic right ventricular dysplasia