title: [Arrhythmogenic right ventricular myocardiopathy. A study of a family group].
abstract: In this paper, we report the cases of 4 male patients (mean age 32.7 yr) with right-ventricular dysplasia, that occurred in familial form. Diagnosis was possible according to the clinical features and to the electrocardiographic, echocardiographic and scintigraphic criteria; at Holter monitoring recorded ventricular arrhythmias with left bundle branch block configuration, associated to sinus arrhythmias (sino-auricular block). In two patients a sustained ventricular tachycardia was recorded. The patients are living and a fourteen-year-old subject is without heart disease. From our data we conclude that: the most serious clinical features occur in extensive form with right atrial and left-ventricle involvement; the clinical features were manifested at a young age and then got worse. A dominant autosomic inheritance is observed (only males were affected).
authors.affiliation: Divisione di Cardiologia, Ospedale di Massa.
authors.forename: W
authors.initials: W
authors.lastname: Serra
authors.forename: S
authors.initials: S
authors.lastname: Giustiniani
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D009202:Cardiomyopathies / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D004452:Echocardiography; D004562:Electrocardiography; D005192:Family Health; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011875:Radionuclide Angiography; D012189:Retrospective Studies
vernacular_title: Miocardiopatia aritmogena del ventricolo destro. Studio di un gruppo famigliare.