Electrical storm in children

Electrical storm (ES) presents a difficult management problem that has predominantly been described in adults and there are limited published data relating to children. We set out to characterize ES in children to assist management based on published literature and own institutional experience. We retrospectively analyzed the records of children presenting with ES to our institution between July 2001 and July 2011 and conducted a systematic literature review. Four children were identified (median age: 5.7 years, range: 3.3-9.6 years, one male). Each ES was of different character and different management strategies were used. All patients were alive at a median follow-up of 5.7 years and all had received implantable cardioverter-defibrillators. Two patients were felt to have catecholaminergic polymorphic ventricular tachycardia, one possible long QT syndrome and one the "short-coupled" variant of torsades de pointes. At least three of our four patients had possible iatrogenic contribution to their ES. Forty-seven cases of ES in children with variable management strategies were identified from the published literature. ES is a rare medical emergency in children with multiple etiologies requiring individualized management.