[Familial arrhythmogenic dysplasia of the right ventricle. Observation of 3 cases].
A family with "arrhythmogenic right ventricular dysplasia (ARVD)" is described. ARVD is pathologically characterised by a partial or total degeneration of the right ventricular myocardium, replaced by fatty and fibrous tissue. This causes dangerous ventricular arrhythmias or congestive heart failure in infancy. About the three described patients, a sixteen-year-old subject died suddenly, and his anatomical and histopathological reports have been presented. Familiarity, rarely treated in literature, and the presence, in the died subject, of a damage of the left ventricle too, are the peculiar characteristic of these "ARVD" cases. The importance about instrumental non-invasive studies has been underlined in order to know early "ARVD", which can be deceptive clinically. The authors suppose there is surely, at the beginning of "dysplasia", a genetic alteration of the right cardiac musculature, but acquired noxae could insert afterwards, on this genetic basis.
D000293:Adolescent; D001145:Arrhythmias, Cardiac / Q000196:embryology / Q000235:genetics* / Q000473:pathology; D006332:Cardiomegaly / Q000175:diagnosis; D004452:Echocardiography; D004562:Electrocardiography; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*
Displasia aritmogena del ventricolo destro ad incidenza familiare. Osservazioni di tre casi.