Arrhythmogenic Right Ventricular Cardiomyopathy: a case report

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease, often familial, characterized pathologically by fibrofatty replacement of the right ventricular myocardium, resulting in thinning of the ventricle. ARVC is an important cause of sudden death in individuals less than 30 years of age and has been found in up to 20% of sudden deaths in young people. A 21-year-old male collapsed to the ground while playing with his friends for about 10 minutes time. There was no history of trauma prior to the collapse. He had a mild upper respiratory tract infection three days prior, however he was not on any medication at the time of this incident. He was immediately taken to the nearby hospital where he was found to be in cardiac arrest. Post-mortem examination revealed an enlarged heart (heart weight 545 g). The right ventricular wall thickness was 6 mm. Coronary arteries were normal in configuration with a left dominant circulation. The right and left coronary ostiae were patent. Coronaries were free from atherosclerotic narrowing. Sections from the right ventricular myocardium showed variable, predominantly epicardial fibrosis throughout and transmural infiltration of adipocytes. The cardiomyocytes in these areas showed patchy vacuolation and nuclear hypertrophy. The remaining myocardium was mainly unremarkable. These findings were in keeping with arrythmogenic right ventricular cardiomyopathy. The importance of a thorough knowledge on specific cardiac conditions that can lead to sudden death and the importance in thorough sampling of cardiac muscle in such cases is highlighted here.