Malignant ventricular arrhythmia in systemic sclerosis controlled with an implantable cardioverter defibrillator

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