Brugada syndrome (BrS) is an inherited
cardiac arrhythmia syndrome characterized by ST-segment elevation in right precordial ECG leads and associated with
sudden cardiac death in young adults. The ECG manifestations of BrS are often concealed but can be unmasked by
sodium channel blockers and
fever. Areas covered: Implantation of a cardioverter
defibrillator (ICD) is first-line
therapy for BrS patients presenting with prior
cardiac arrest or documented VT. A pharmacological approach to
therapy is recommended in cases of electrical storm, as an adjunct to ICD and as preventative
therapy. The goal of pharmacological
therapy is to produce an inward shift to counter the genetically-induced outward shift of
ion channel current flowing during the early phases of the ventricular epicardial action potential. This is accomplished by augmentation of ICa using â–¡â–¡adrenergic agents or
phosphodiesterase III inhibitors or via inhibition of Ito.
Radiofrequency ablation of the right ventricular outward flow tract epicardium is effective in suppressing arrhythmogenesis in BrS patients experiencing frequent appropriate ICD-shocks. Expert commentary: Understanding of the pathophysiology and approach to
therapy of BrS has advanced considerably in recent years, but there remains an urgent need for development of cardio-selective and
ion-channel-specific Ito blockers for treatment of BrS.