Brugada Syndrome

Abstract

Abstract-The Brugada syndrome has gained wide recognition throughout the world and today is believed to be responsible for 4% to 12% of all sudden deaths and Ϸ20% of deaths in patients with structurally normal hearts. The incidence of the disease is on the order of 5 per 10 000 inhabitants and, apart from accidents, is the leading cause of death of men under the age of 50 in regions of the world where the inherited syndrome is endemic. This minireview briefly summarizes the progress made over the past decade in our understanding of the clinical, genetic, cellular, ionic, and molecular aspects of this disease. Key Words ventricular tachycardia Ⅲ ventricular fibrillation Ⅲ arrhythmia Ⅲ sudden death T his year marks the 10th anniversary of the initial description by Pedro and Josep Brugada of an intriguing new clinical entity characterized by an ST-segment elevation in the right precordial ECG leads and a high incidence of sudden death in individuals with structurally normal hearts. 1 The purpose of this minireview is to briefly summarize the progress made over the past decade in our understanding of the clinical, genetic, cellular, ionic, and molecular aspects of this disease.In 1986, a 3-year-old Polish boy was referred to the Brugada brothers after multiple episodes of syncope. His ECG showed an ST-segment elevation in leads V 1 to V 3 . His sister displayed a similar clinical and electrocardiographic profile and died at 2 years of age. In the succeeding years, six additional cases came to their attention, and in 1992 they reported these eight cases as the basis for a new and distinct clinical entity. 1 In 1996, Yan and Antzelevitch 2 highlighted the importance of the ST-segment elevation and apparent right bundlebranch block (RBBB) described by Brugada and Brugada as the basis for a substrate capable of giving rise to malignant arrhythmias, naming it the Brugada syndrome. Kobayashi et al 3 and Miyazaki et al 4 followed suit that same year.The electrocardiographic pattern of ST-segment elevation and inversion of the T wave in the right precordial leads, with and without RBBB, was described as early as 1953. 5 This ECG phenomenon was largely ignored until Martini et al 6 and Aihara et al 7 brought attention to a possible link between this ventricular repolarization abnormality and sudden death. Martini et al 6 maintained that structural or morphological defects contribute importantly to arrhythmogenesis in patients with the Brugada syndrome, whereas Brugada et al 1 excluded this from the diagnosis, stressing the functional basis for the syndrome. Corrado et al 8 subsequently described a subpopulation of arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) patients displaying features of the Brugada syndrome, including ST-segment elevation in V1 to V3 and polymorphic ventricular tachycardia (VT), suggesting that a subgroup of patients with ARVC can display a Brugada-like phenotype during the relatively early stages of the disease.It is noteworthy that arrhythmogenic RV dysplasia/ARVC and Brugada syndromes a...

Frequency of Brugada-Type ECG Pattern (Brugada Sign) in Southern Turkey

“…5,13,15) Junttila, et al 6) reported a ratio of 0.61% in 2479 young males aged between 18-30 years, and 0.55% in 542 individuals aged between 40-60 years. In Japan, the frequency in males has been reported to vary between 0.14-2.41% and 0.04% in females.…”

Electrical heart disease: Genetic and molecular basis of cardiac arrhythmias in normal structural hearts

“…The clinical presentation is heterogeneous, including palpitations, dizziness, syncope and (aborted) sudden death, although many subjects are asymptomatic (44,45). It is believed to cause 4% to 12% of all sudden cardiac deaths and up to 20% among patients without identifiable structural abnormalities in some regions of the world (46). Men have a higher disease prevalence, particularly in regions where Brugada syndrome is endemic (southeast Asia), despite equal genetic transmission between both sexes (47).…”

The Brugada Syndrome-An Update-

“…In 1992, Brugada and Brugada first described 8 patients with a history of aborted sudden cardiac death due to ventricular fibrillation (VF) and a characteristic electrocardiographic (ECG) pattern, consisting of right bundle branch block (RBBB) and ST-segment elevation in the right precordial leads (V1-V3) as a distinct clinical entity (1)(2)(3)(4)(5)(6)(7)(8). This syndrome is not related to acute ischemia, electrolyte abnormalities or structural heart diseases (6).…”