Pitfalls in arrhythmogenic left ventricular cardiomyopathy (ALVC). A review of the literature with considerations on a single case of sudden death in a juvenile athlete

Sudden cardiac death (SCD) in young athletes represents a challenging issue in forensic practice. The pathologist is frequently asked to establish the cause of death basing upon anatomical findings and to evaluate the role of the physician in preparticipation evaluation (PPE) and eligibility decision. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of SCD during sport activity. However, in the last few years, forms with predominant or even isolated involvement of the left ventricle (LV) have progressively been correlated with a high risk of SCD. We present a case of SCD in an apparently healthy 19-year-old semi-professional football player. Annual PPEs performed in accordance with international and Italian recommendations, were unremarkable. At autopsy, a 1-cm area of subepicardial fibro-fatty replacement was observed at the postero-lateral wall of the LV. The finding was diagnostic of arrhythmogenic left ventricular cardiomyopathy (ALVC). A review of this rare pathology has been performed under a forensic perspective, focusing on the evaluation of the medico-legal responsibility of the physician in the PPE and on the morphological aspects of the disease. Current diagnostic criteria and recommendations result to be focused on the right ventricular pattern, with a risk of misdiagnosis for isolated LV forms. Furthermore, few detailed autopsies cases concerning ALVC have been published. There is a need, therefore, to study this rare disease with a careful and revised approach.