Cardiac arrest from arrhythmogenic right ventricular dysplasia in an adolescent.

We report the case of a 16-year-old girl who experienced sudden cardiac arr est from ventricular fibrillation, complicating an arrhythmogenic right ven tricular dysplasia, a rare heart muscle disorder, occurring typically in yo ung adults, characterized by a fibrofatty replacement of the right Ventricu lar myocardium. Symptomatic ventricular arrhythmias are frequent, and sudde n death has been reported. In our case, diagnosis of arrhythmogenic dysplas ia was based on the association of one major criterion and two minor criter ia as suggested by the relevant task force. in contrast with most other rep orts, the chest ECG did not display the typical features. An automatic tran svenous pectoral cardioverter-defibrillator was implanted. The authors emph asise that juvenile forms are more exposed to ventricular fibrillation and sudden cardiac death, and consequently require the early detection of the d isease. Family cases have been described and the occurrence in one individu al must lead to investigations in the relatives. (C) 1998 Elsevier, Paris.