Citation

Paoloni P, Cardinali L, Mennecozzi M, Capone P. Minerva Cardioangiol. 1995; 43(5): 231-235.

Vernacular Title

Displasia aritmogena del ventricolo destro. Descrizione di un caso di

Affiliation

Servizio di Cardiologia, Ospedale Civile, Fermo, Ascoli Piceno.

Copyright

(Copyright © 1995, Edizioni Minerva Medica)

DOI

unavailable

PMID

7478047

Abstract

Arrhythmogenic right ventricular dysplasia is a pathologic condition of unknown origin that primarily affects the right ventricle free wall characterized by progressive atrophy with fibrous-fatty substitution of the myocardium. The patient affected may present in childhood or as an adult with cardiomegaly or/and ventricular arrhythmias with a left bundle branch block configuration. The reports of familial occurrence suggest the etiologic role of a genetic defect with autosomal dominance and variable expression and penetrance, an alternative, myocarditis (toxic or infectious) is a possible cause. The aim of the present study is an non-invasive instrumental evaluation of the family of a young patient affected by arrhythmogenic right ventricle dysplasia. The authors evaluated 10 patients, 7 women and 3 men, all asymptomatic, with electrocardiography, exercise stress test on a bicycle ergometer, 24-hour Holter monitoring and two-dimensional echocardiography. A patient had certain ARVD diagnosis, while in the other 9 patients all the instrumental investigations were normal. This observation seem to confirm that ARVD may be familial and justify a systematic in-depth familial study in all demonstrated cases of ARVD and the follow-up of all patients identified who had arrhythmias or isolated right ventricular morphologic anomalies.

Language: it