Arrhytmogenic cardiomyopathy is a disorder characterized by fatty and fibrous replacement of the ventricular myocardium. Clinical manifestations consist of ventricular arrhythmias, syncope, progressive heart failure and occasional sudden deaths. We report the case of a 19-year old football player who collapsed immediately after beginning warming up exercise before a football training. ECG monitoring highlighted ventricular fibrillation and resuscitation attempts were unsuccessful. Past history was positive only for a hypertensive episode at age 13 and at the annual sport evaluation he was declared fit for sporting activity. Family history disclosed a myocardial infarction in a paternal uncle. Autopsy was performed according to guidelines for sudden deaths and it disclosed pulmonary oedems and congestion, a 425-gram heart weight and coronary arteries free from atherosclerotic disease. A 1 cm area of fibro-fatty subepicardial replacement was detected at the antero-lateral wall of the left ventricle and three foci of myocardial disarray surrounded by fibrosis were observed in the interventricular septum. No inflammatory infiltrate was noted and the right ventricle was unremarkable. Toxicology was negative, whereas genetic analysis was not available. The cause of the death was attributed to a fatal ventricular arrhythmia due to a left-dominant arrhythmogenic cardiomyopathy. Arrhythmogenic cardiomyopathy has been typically classified into a right ventricular (classical form) and biventricular pattern. The left-dominant phenotype represents the 5% of the overall cases and most of the literature consists of case reports. Main anatomical and clinical features of this disease are described in this communication, underlying that clinical manifestation display more often earlier than prominent morphological alterations.
