Update on the Diagnosis and Management of Brugada Syndrome

Abstract

Brugada Syndrome (BrS) is an autosomal dominant channelopathy with variable penetrance affecting the sodium channel. It reduces the transport of sodium ions essential for proper generation of the cardiac action potential. The resulting inhomogeneous repolarisation in areas of the RV epicardium causes malignant ventricular arrhythmias. BrS is diagnosed by typical cove shaped ST elevation of > 2 mm in ≥ 1 RV precordial lead V1, V2 occurring spontaneously or after provocative drug test with IV administration of Class 1 antiarrhythmic drug such as flecainide or ajmaline. The incidence of BrS is variable being higher in South East Asians and is generally quoted as 1:2000. It is responsible for up..