Brugada Syndrome (BrS) is a genetic disorder characterised by right bundle branch block pattern with ST segment elevation and T wave inversion in right precordial leads. Life threatening ventricular arrhythmias are the hallmark of BrS. The disorder was described by Brugada brothers (Pedro, Joseph and Ramon) in 1992. Later on the genetic defect was localised to the sodium channel gene SCN5A. Patients with BrS may sometimes present with electrical storm. Medical treatment is generally unsatisfactory for BrS. Drugs like quinidine and cilostazol have been useful in reducing the number of episodes of ventricular tachyarrhythmia. Those with previous cardiac arrest, especially with a family history of sudden cardiac death need to be protected by an implantable cardioverter defibrillator (ICD). ICDs can detect and treat ventricular tachycardia either by overdrive pacing suppression or cardioversion.
Image courtesy: Francis J, Sajeev CG, Venugopal K. Brugada Syndrome: The Enigma. Calicut Medical Journal 2003;1(1):e3
Sometimes the classical Brugada pattern in the ECG may not be obvious and has to be brought out by flecainide challenge.