Pediatric electrical storm: clinical characteristics, management, and outcomes in a multicenter cohort

Background

Electrical storm (ES) in children is an exceptional yet life-threatening arrhythmic emergency. Data on its management and prognosis remain scarce, and therapeutic strategies are often extrapolated from adult practice.

Purpose

To characterize pediatric ES in terms of underlying diseases, acute management, and outcomes, and to identify rescue interventions associated with survival and recurrence.

Methods

We retrospectively analyzed 49 pediatric patients (median age 10 [3–15] years, 73.5 % male) admitted for sustained ventricular arrhythmia or ES across participating French centers (2015–2025). Clinical presentation, in-hospital management, and follow-up were collected from institutional databases and analyzed descriptively.

Results

Underlying heart disease was identified in 77 %, including dilated (12.5 %) and hypertrophic cardiomyopathy (10.4 %), arrhythmogenic right ventricular dysplasia (10.4 %), and long-QT syndrome (12.5 %); 19 % had idiopathic VT/VF. ES was the initial diagnosis in 48 %. Monomorphic VT occurred in 55 % and polymorphic VT/VF in 45 %. Syncope was reported in 72 %, and 59 % were hemodynamically unstable, including 23 % with cardiogenic shock.

Amiodarone and β-blockers were the most frequent therapies (51 % and 53 %), with adjunctive lidocaine in 16 % and calcium-channel blockers in 12 %. Deep sedation and catecholamines were required in 58 % and 54 %, respectively. Catheter ablation was attempted in 31 %, and sympathetic neuromodulation in 8 %. Three patients (6 %) underwent urgent heart transplantation. In-hospital mortality was 6 %.

During a median 1.9 [0.6–5.8] years of follow-up, ventricular arrhythmia recurred in 29 % and ES in 9 %, while overall mortality reached 4 %.

Conclusions

Pediatric electrical storm is associated with high acute morbidity and frequent hemodynamic compromise. Pharmacological therapy alone is rarely sufficient; ablation and neuromodulation are underused but feasible even in unstable patients. Heart transplantation remains a viable rescue option in refractory cases. Dedicated pediatric algorithms and earlier escalation strategies are needed to improve outcomes in this fragile population.Overall survivalSurvival free from VTVF