The Brugada syndrome.
The Brugada syndrome.
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DOI：
10.1007/978-1-59259-362-0_19
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发表时间：
2002
影响因子：
2.3
通讯作者：
G. Naccarelli;Charles Antzelevitch;D. Wolbrette;J. Luck
中科院分区：
文献类型：
--
作者：
G. Naccarelli;Charles Antzelevitch;D. Wolbrette;J. Luck
In 1992 a syndrome consisting of syncope episodes and/or sudden death in patients with a structurally normal heart and a characteristic electrocardiogram (ECG) displaying a pattern resembling right bundle branch block with ST segment elevation in leads V1 to V3 was described. The disease is genetically determined with an autosomal dominant pattern of transmission in 50% of the familial cases. Several different mutations have been identified affecting the structure and the function of the sodium channel gene SCN5A. These mutations result in loss of function of the sodium channel. The syndrome appears ubiquitous. The incidence of the disease is difficult to estimate worldwide, but it may cause 4 to 10 sudden deaths per 10,000 inhabitants per year in areas like Thailand and Laos. In these countries, the disease represents the most frequent cause of natural death in young adults. It is estimated that 20 to 50% of sudden deaths in patients with a normal heart result from this syndrome. The disease has been linked to the sudden infant death syndrome and to the sudden unexpected death syndrome by showing that the electrocardiogram and mutations are the same as in Brugada syndrome. The diagnosis is easily made by means of the ECG when it is typical. There exist, however, patients with concealed and intermittent electrocardiographic forms that make the diagnosis difficult. The ECG can be modulated by changes in autonomic balance, body temperature, glucose level, and the administration of drugs like antiarrhythmics, but also neuroleptic and antimalaria drugs.
