The Brugada Syndrome: Is an Implantable Cardioverter Defibrillator the Only Therapeutic Option?

In 1992 two Spanish cardiologist brothers, Pedro and Josep Brugada reported eight patients with aborted cardiac arrest and no demonstrable heart disease who exhibited in sinus rhythm right bundle branch block (RBBB) with prominent STsegment elevation in precordial leads V 1‐V3. 1 Despite initial controversy about the diagnosis, especially concerning the possibility of a subtle arrhythmogenic right ventricular dysplasia, the repeated lack of right ventricular involvement along with consistent clinical, electrocardiographic (ECG), and electrophysiological features convinced the cardiological community that the Brugada syndrome was actually a new and important cause of sudden cardiac death (SCD) in ostensibly healthy patients. 2 During the last decade an increased awareness among physicians has resulted in a growing number of patients reported worldwide. In 1998, Chen et al. 3 were first to establish that the Brugada syndrome was a genetic disease with an autosomal dominant pattern of transmission. These investigators described mutations all affecting the cardiac sodium channel SCN5A on chromosome 3. More recently, a novel gene locus on chromosome 3, distinct from SCN5A has been identified. 4 The genetic pattern of transmission of the disease has lead to the increased detection of asymptomatic patients affected by the disease among families of cardiac ar