[Cardiac arrest in an adolescent with arrhythmogenic right ventricular dysplasia]

We report the case of a 16-year-old girl who experienced sudden cardiac arrest from ventricular fibrillation, complicating an arrhythmogenic right ventricular dysplasia, a rare heart muscle disorder, occurring typically in young adults, characterized by a fibrofatty replacement of the right ventricular myocardium. Symptomatic ventricular arrhythmias are frequent, and sudden death has been reported. In our case, diagnosis of arrhythmogenic dysplasia was based on the association of one major criterion and two minor criteria as suggested by the relevant task force. In contrast with most other reports, the chest ECG did not display the typical features. An automatic transvenous pectoral cardioverter-defibrillator was implanted. The authors emphasise that juvenile forms are more exposed to ventricular fibrillation and sudden cardiac death, and consequently require the early detection of the disease. Family cases have been described and the occurrence in one individual must lead to investigations in the relatives.