Electrical storms in Brugada syndrome successfully treated with isoproterenol infusion and quinidine orally

Abstract

Brugada syndrome is an inherited cardiac disease and is associated with a peculiar pattern on the electrocardiogram and an increased risk of sudden death. Electrical storm is a malignant but rare phenomenon in symptomatic patients with Brugada syndrome. We describe a patient who presented with repetitive ICD discharges during two episodes of recurrent VF. After the initiation of isoproterenol infusion and oral quinidine, the ventricular tachyarrhythmias were successfully suppressed. (Neth Heart J 2007;15:151-4.).

A Case of Brugada Syndrome Presenting With Incessant Polymorphic Ventricular Tachycardia

“…This effect is more prominent in the right ventricle and is reflected in the right precordial ECG leads. 3,8 Furthermore, it is felt that further shift in the balance of currents leads to heterogeneous loss of the action potential dome throughout the epicardium. This creates an arrhythmogenic substrate from the development of a marked transmural and epicardial dispersion of repolarization and refractoriness allowing for reentrant arrhythmias to occur.…”

“…This creates an arrhythmogenic substrate from the development of a marked transmural and epicardial dispersion of repolarization and refractoriness allowing for reentrant arrhythmias to occur. 3,8 There are 3 types of ECG manifestations that are recognized. The type 1 pattern is diagnostic of Brugada syndrome and is characterized by coved ST-segment elevation greater than 2 mm and followed by a negative T wave (Figure 1).…”

“…Thus, tedisamil may be more potent than quinidine in Brugada syndrome. 2,3,6 Other agents that can be considered include β-adrenergic agonists (isoproterenol) and phosphodiesterase III inhibitors (cilostazol). Isoproterenol stimulates cyclic adenosine monophosphate formation which boosts the ICa current.…”

“…2 Although the occurrence of ventricular arrhythmias is high in this syndrome, a ventricular tachycardia/ventricular fibrillation (VT/VF) storm, characterized by more than 3 episodes in a 24 hour period, is rare and infrequently reported. 3 Because no pharmacological therapy has been established for this syndrome, management of a VT/VF storm in this setting remains unclear. In case reports, isoproterenol, quinidine, and cilostazol have been reported to be effective treatments.…”

Antiarrhythmic drugs, patients, and the pharmaceutical industry: value for patients, physicians, pharmacists or shareholders?

“…4 Diagnosed as symptomatic Brugada syndrome in 2004, he received an ICD. After successful defibrillation in 2005, he presented with an arrhythmic storm in 2006, which was successfully treated with isoproterenol and quinidine.…”