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  • Title: [Arrhythmogenic right ventricular myocardiopathy. A study of a family group].
    Author: Serra W, Giustiniani S.
    Journal: Minerva Cardioangiol; 1991 May; 39(5):185-90. PubMed ID: 1956545.
    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).
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