Introduction: 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 I_Ca using □□adrenergic agents or phosphodiesterase III inhibitors or via inhibition of I_to. 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 I_to blockers for treatment of BrS.