La cardiomiopatia aritmogena del ventricolo dx: aspetti clinici e medico-legali

The arrhythmogenic right ventricular cardiomyopathy is a primitive disease caused by defects of the genes coding the proteins that keep the cardiomyocites jointed at cellular junctions (desmosomal disease), and it is characterized by the presence of structural alterations (fibrofatty infiltration with areas of surviving myocytes, typically located at the angles of the “triangle of dysplasia” at the infero-apical and infundibular walls) and functional alterations of the right ventricle, with related electric instability and ventricular arrhythmias, to the point of ventricle fibrillation, with a risk of sudden death. In fact, the development of fibrofatty scans, either diffused or regional, with adipose fibers replacement and dilation of the right ventricle, promotes the appearance of re-entry circuits which are the origin of ventricular arrhythmias. ARVC is a hereditary disease genetically determined, with dominant autosomic transmission, and with incomplete penetration. After carrying out a complete review of modern techniques used to evaluate cardiac function, the research focuses on the specific aspects of the disease from a forensic medicine perspective with particular emphasis – due to the disposition of the disease – on two basic tests: echocardiography and cardiovascular magnetic resonance (CMR). With regards to the forensic medicine aspects, and taking into consideration that the disease is one of the main causes of sudden cardiac death (SCD) in young athletes, prime aspects of medical professionals’ responsibility due to lack of a diagnosis of the disease are investigated, as well as the lack of prophylaxis of a malignant arrhythmia with implantable cardioverter defibrillator (ICD). Moreover, since effort is a possible trigger for ventricular arrhythmias in subjects affected by ARVC, possible responsibilities of physicians issuing certificates of sporting fitness are considered. Lastly, dealing with a pathology that affects primarily youth and frequently generates damages that have to be evaluated in a ...