Brugada Syndrome

Abstract

In 1986, two young brothers who survived documented cardiac arrests exhibited a specific electrocardiographic pattern providing a link to sudden death. Similar observations in other individuals led to the original description in 1992 of the syndrome consisting of right bundle branch block, ST-segment elevation in leads V1 to V3, and sudden death with structurally normal heart—ie, the Brugada syndrome.1 This syndrome has stimulated research in many centers. What before was an electrocardiographic pattern resembling early repolarization has now become a marker for increased risk of sudden death. Fortunately, this observation coincided with an outpouring of molecular research in cardiology and with the success already encountered in identifying the genetic basis of other arrhythmic diseases. Brugada syndrome likely will continue to benefit from the application of genomics. In just 10 years, the disease has become widely recognized, and researchers in multiple disciplines in basic and clinical research have come together to decipher its pathogenesis. See p 3081 The most common presentation is a middle-aged man, with the electrocardiographic blueprint, no structural heart disease, and a documented history of ventricular fibrillation.2 In 1997, Nademanee et al3 linked Brugada syndrome to sudden unexpected death syndrome (SUDS) in Southeast Asia. SUDS has been recognized since the 1980s, when the Centers for Disease Control reported a higher than usual incidence of sudden death in individuals from that geographic area.4 The individuals usually die during their sleep from ventricular tachyarrhythmias. In some countries, SUDS is an important cause of death in young males, second only to car accidents. The electrocardiographic pattern is the same as that seen in Brugada syndrome, indicating that SUDS and Brugada syndrome are similar diseases, if not the same.3 A sex difference in Brugada syndrome is obvious in Southeast Asia, where only males are …