Prevalence of the Brugada syndrome in an apparently healthy population

Autor: Jean-Luc Rey, Jean-Sylvain Hermida, Jean-Claude Quiret, Geneviève Jarry, Fadi Bou Aoun, Jean-Luc Lemoine
Rok vydání: 2000
Předmět:
Zdroj: The American Journal of Cardiology. 86:91-94
ISSN: 0002-9149
DOI: 10.1016/s0002-9149(00)00835-3
Popis: The syndrome associating right bundle branch block, ST-segment elevation in leads V1, V2 to V3, and aborted sudden cardiac death due to ventricular fibrillation was first reported in 1992 by Brugada and Brugada. Mutations in SCN5A, a gene encoding the cardiac sodium channel, have been evidenced in some patients. Usually, the ST-segment elevation has a coved form, but change into a saddleback form or complete normalization appear to be common (30%), leading to underdiagnosis of the syndrome. The right bundle branch shows varying degrees of conduction disturbances, and in some cases the right bundle branch block can be absent. In patients in whom the syndrome is suspected despite a normal electrocardiogram, ST-segment elevation can be reproduced by ajmaline. According to Brugada, asymptomatic patients recognized at random or discovered in a family study, have a 27% incidence of arrhythmic events during a mean follow-up of 34 months. A coved ST-segment elevation has been also observed in patients with arrhythmogenic right ventricular dysplasia, in Chagas or Steinert diseases, and in mediastinal tumors. Also, the early repolarization syndrome may show some electrocardiographic similarities with the saddleback type of Brugada syndrome. Consequently, the clinical significance of the different types of ST-segment elevations may appear uncertain in asymptomatic patients, especially in a patient with the isolated saddleback type. The disease is the most common cause of sudden cardiac death in young persons in south Asia. In Japan, the prevalence of electrocardiograms compatible with the syndrome is 0.05%. In Europe, the disease has been recognized recently and there are no data available on its prevalence. Currently, the implantable cardiac defibrillator is the only effective treatment and is indicated in symptomatic patients; it should be considered in asymptomatic patients in whom polymorphic sustained ventricular tachycardia is inducible during electrophysiologic study. This study assesses the prevalence of the electrocardiographic patterns of the Brugada syndrome in an apparently healthy, asymptomatic, European population, and determines whether these patterns were associated with increased cardiac mortality.
Databáze: OpenAIRE
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