MALIGNANT VENTRICULAR ARRHYTHMIA IN SYSTEMIC-SCLEROSIS CONTROLLED WITH AN IMPLANTABLE CARDIOVERTER-DEFIBRILLATOR

The prognosis of systemic sclerosis (SSc) is poor, and a significant n umber of patients suffer sudden death, probably related to malignant v entricular arrhythmias for which there is no reliable drug treatment. We describe a patient with SSc who had 2 documented episodes of ventri cular fibrillation, that reverted after electrical defibrillation. An electrophysiological study performed after 3 weeks of oral loading wit h amiodarone 1200 mg/day, demonstrated the induction of unstable susta ined syncopal ventricular tachycardia. She has been successfully contr olled with the implant of a 3rd generation implantable cardioverter de fibrillator. We suggest that this treatment should be seriously consid ered in those patients with SSc and malignant ventricular arrhythmias unresponsive to drug therapy.