Electrical Storm in Patients with Implantable Cardioverter-defibrillators: A Practical Overview

Electrical storm (ES) is an increasingly common medical emergency characterized by clustered episodes of sustained ventricular arrhythmias (VAs) that lead to repeated appropriate implantable cardioverter-defibrillator (ICD) therapies. A diagnosis of ES can be made with the occurrence of three or more sustained episodes of VAs, or of three or more appropriate ICD therapies within 24 hours in patients with implanted devices. ES is associated with poor outcomes in patients with structural heart disease, particularly those with severe left ventricular dysfunction. In large clinical trials involving patients with ICDs for primary and secondary prevention, ES appears to be a predictor of cardiac death, with notably higher rates of mortality soon after the event. ES management is challenging and requires special medical attention with accurate patient risk stratification and a multidisciplinary approach that includes the use of pharmacologic therapies such as antiarrhythmic drugs (AADs) and interventional approaches like catheter ablation, surgical ablation, or sympathetic neuromodulation. Initial management involves determining and addressing the underlying ischemia, any electrolyte imbalances, and/or other causative factors. Hemodynamic support needs to be considered in high-risk patients with unstable VAs or those with severe comorbidities such as low left ventricular ejection fraction, advanced New York Heart Association class, and/or chronic pulmonary disease. Following the acute phase of ES, treatment should shift towards maximizing therapeutic efforts to address heart failure, performing revascularization, and preventing subsequent VAs. In the present manuscript, we offer an overview of the most relevant clinical aspects of ES with regard to novel therapeutic strategies.