title: [Right ventricular dysplasia and sudden death in young people].
abstract: In this study two autoptic cases of right ventricular dysplasia, observed in young and asymptomatic subjects who died suddenly are reported. In these patients, the "primary" myocardial atrophy involved the right ventricle, the right atrium and the conduction system: the sinoatrial node and the internodal pathways (in both cases) and the hisian bifurcation (only in the second case). The primary ventricular dysplasia was always associated with "secondary" transmural hyperplasia of the sub-epicardial fat tissue except for the anterior wall of the pulmonary infundibulum. According to the findings observed, we think that: 1) in all cases of right ventricular dysplasia a careful examination of the conduction system must be performed due to the fact that the lethal arrhythmias can be not only "hyperkinetic" but also "hypokinetic" arrhythmias; 2) right ventricular dysplasia is a dysplastic-congenital disease and transmural fatty hyperplasia depends both on "primary" myocardial atrophy and on the presence of sub-epicardial fat tissue; the latter is related to the age of the patient.
authors.affiliation: Istituto di Anatomia ed Istologia Patologica, Università degli Studi di Palermo.
authors.forename: E
authors.initials: E
authors.lastname: Maresi
authors.forename: N
authors.initials: N
authors.lastname: Albano
authors.forename: P
authors.initials: P
authors.lastname: Procaccianti
authors.forename: G
authors.initials: G
authors.lastname: Campesi
mesh_terms: D000328:Adult; D009202:Cardiomyopathies / Q000473:pathology*; D003645:Death, Sudden / Q000209:etiology*; D005260:Female; D006329:Heart Conduction System; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D009206:Myocardium / Q000473:pathology; D012849:Sinoatrial Node / Q000473:pathology
vernacular_title: Displasia del ventricolo destro e morte improvvisa giovanile.