Electrical Storm in Children

Abstract

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.

Optimal antiarrhythmic drug therapy for electrical storm

“…This effect correlates to prior data from acute myocardial infarction patients in the β-blocker Heart Attack Trial in which β-blocker reduced mortality largely from prevention of ventricular tachyarrhythmias[62]. For channelopathies, such as catecholaminergic polymorphic VT, β-blockade also is the mainstay of treatment[7].…”

“…More typically, the definition for ES includes 3 or more separate episodes of ventricular tachyarrhythmias, whether untreated or treated with anti-tachycardia pacing or shocks ( Fig. 1 )[7]-[9]. Hemodynamic instability is not required to be associated with ES.…”

“…Ischemia or worsening of heart failure predominates as the etiology in adults, while congenital heart disease and primary electrical disease are more common in children, who have a significantly lower frequency of ES overall compared to adults1,2,7,8,[12]-[15]. Common and uncommon causes of ES are listed in Table 1.…”

Electrical storm in an adolescent with arrhythmogenic right ventricle cardiomyopathy treated with cardiac transplantation

“…Arrhythmogenic right ventricle dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by fibro fatty replacement of the right and less frequently left ventricle ( 1 , 2 ). Ventricular arrhythmias requiring implantable cardioverter defibrillator (ICD) are common in patients with ARVD/C and electrical storm (≥3 life-threatening ventricular arrhythmia within 24-hour period) resulting in ICD discharges is a major cause of morbidity and mortality ( 3 ). Radiofrequency ablation with three-dimensional (3-D) mapping and navigation systems has been recently advocated as a preferred treatment for recurrent ventricular arrhythmias ( 4 ).…”

Bolus potassium in frustrated ventricular fibrillation storm

“…The electrical storm is a syndrome involving repeated episodes of ventricular tachycardia (VT) or ventricular fibrillation (VF) . Usually three episodes or more within 24 hours . The predominant etiology in adults is ischemia or worsening heart failure.…”