Brugada Syndrome: Report of the Second Consensus Conference

Abstract

Abstract-Since its introduction as a clinical entity in 1992, the Brugada syndrome has progressed from being a rare disease to one that is second only to automobile accidents as a cause of death among young adults in some countries. Electrocardiographically characterized by a distinct ST-segment elevation in the right precordial leads, the syndrome is associated with a high risk for sudden cardiac death in young and otherwise healthy adults, and less frequently in infants and children. Patients with a spontaneously appearing Brugada ECG have a high risk for sudden arrhythmic death secondary to ventricular tachycardia/fibrillation. The ECG manifestations of Brugada syndrome are often dynamic or concealed and may be unmasked or modulated by sodium channel blockers, a febrile state, vagotonic agents, ␣-adrenergic agonists, ␤-adrenergic blockers, tricyclic or tetracyclic antidepressants, a combination of glucose and insulin, hypo-and hyperkalemia, hypercalcemia, and alcohol and cocaine toxicity. In recent years, an exponential rise in the number of reported cases and a striking proliferation of articles defining the clinical, genetic, cellular, ionic, and molecular aspects of the disease have occurred.

Long‐Term Prognosis of Brugada‐Type ECG and ECG With Atypical ST‐Segment Elevation in the Right Precordial Leads Over 20 Years: Results From the Circulatory Risk in Communities Study (CIRCS)

“…The types of BrS were classified according to the ECG criteria described in the report of the second consensus conference, which defined type 1 as characterized by a coved‐type J point elevation ≥2 mm (0.2 mV) and non–type 1 as characterized by type 2 or 3 BrS ECG, that is, a saddleback‐type J point elevation ≥2 mm (0.2 mV) 3. Patients who had ECGs with J point elevation ≥0.2 mV in the right precordial leads and non‐BrS ECG were categorized as having STERP.…”

“…Brugada syndrome is a hereditary arrhythmogenic disease characterized by accentuated ST‐segment elevation (type 1) in the right precordial leads (V 1 through V 3 ) and increased risk of sudden cardiac death (SCD) without structural heart disease 1, 2, 3. The data from previous studies vary regarding prevalence and prognosis for patients with Brugada‐type ECG (BrS) because of different populations (eg, general population, office workers),4, 5, 6, 7, 8, 9 different ECG definitions,5, 6, 9, 10, 11, 12 relatively short‐term follow‐up,5, 6, 9, 10, 12, 13 or poorly defined SCD 5, 6, 7, 8, 10, 11, 13, 14…”

Sodium channel blockade unmasked Brugada electrocardiographic pattern in a patient with complete right bundle branch block and early repolarization in the lateral leads

“…After an injection of a sodium channel blocker, the J waves in BrS become more prominent, while those in ERS either do not change or become attenuated. The Brugada Consensus Report proposed that a sodium channel blockade is required to diagnose BrS in the absence of a coved‐type ST‐segment elevation 2. Moreover, some reports have demonstrated that the J waves in BrS are concealed by CRBBB 8, 9.…”

“…However, the effects of sodium channel blockers on the J waves differ greatly between ERS and BrS 2.…”

“…Early repolarization syndrome (ERS) and Brugada syndrome (BrS) share many electrocardiographic and clinical features, and recently have been collectively grouped as J wave syndrome 1, 2. However, the effects of sodium channel blockers on the J waves differ greatly between ERS and BrS 2.…”