TY  - JOUR
PY  - 1995//
TI  - Arrhythmogenic right ventricular dysplasia. Report of a familial case
JO  - Minerva cardioangiologica
A1  - Paoloni, P.
A1  - Cardinali, L.
A1  - Mennecozzi, M.
A1  - Capone, P.
SP  - 231
EP  - 235
VL  - 43
IS  - 5
N2  - 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.<p /> <p>Language: it</p>
LA  - it
SN  - 0026-4725
UR  - http://dx.doi.org/
ID  - ref1
ER  -