Arrhythmogenic right ventricular cardiomyopathy - cause of sudden death in young people

D. Butcovan, C. Amalinei, C. Grigoriu

Abstract: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is a heart muscle disease pathologically characterized by progressive fibro fatty replacement of myocytes, initially involving the right ventricle. We report the case of a 21 years-old patient, with no cardiac history, who died suddenly during an intense exercise. The heart was completely examined macroscopically. Full-thickness blocks of myocardium were removed for histologic examination from the right and left ventricles and the ventricular septum. Myocardial sections were stained with hematoxylin–eosin, van Gieson and Masson trichrome dies. According to gross inspection and histologic examination, the right ventricle disclosed severe replacement of the wall thickness by fibro-fatty tissue except for a subendocardial layer and trabeculae which showed surviving myocardium with hypertrophy, degenerative changes and increased interstitial fibrosis. The free left myocardial wall showed patchy replacement fibrosis and scattered interstitial collections of mononuclear cells. ARVC, as a common cause of sudden death of the young adults, is not enough explored. Under these circumstances, the diagnosis must trigger investigations, not only for disease understanding, but also for preventing unexpected fatal clinical situations.
Keywords: arrhythmia, cardiomyopathy, sudden death