Brugada P, Brugada R, Brugada J, Geelen P
Cardiovascular Research and Teaching Institute, Aalst, Belgium.
Am J Cardiol. 1999 Mar 11;83(5B):98D-100D. doi: 10.1016/s0002-9149(98)01009-1.
About 10-20% of patients dying suddenly and unexpectedly do not have structural heart disease. The major causes of sudden death in this population are acute ischemia, the syndrome of right bundle branch block, and ST-elevation from V1 to V3, the long QT-syndrome, and the Wolff-Parkinson-While syndrome. In some patients, none of these syndromes can be recognized and ventricular fibrillation is classified as idiopathic. There are good preventive and therapeutic methods against acute ischemia and there are also curative treatments for the Wolff-Parkinson-White syndrome. Patients with idiopathic ventricular fibrillation cannot be recognized beforehand. However, there are electrocardiographic and genetic markers for the Brugada syndrome and the long QT syndrome. It is, therefore, justified to discuss the possible role of the prophylactic defibrillator to prevent sudden death in these 2 syndromes for which no effective treatment exists. Patients with Brugada syndrome have a high incidence of sudden death, and prophylactic defibrillators are indicated in patients with inducible arrhythmias at electrophysiologic study, irrespective of symptoms. On the contrary, the incidence of sudden death in the long QT syndrome is very low, making prophylactic defibrillator implantation not cost-effective.
约10% - 20%突然意外死亡的患者并无结构性心脏病。这部分人群猝死的主要原因包括急性缺血、右束支传导阻滞综合征、V1至V3导联ST段抬高、长QT综合征以及预激综合征。在一些患者中，无法识别出这些综合征，室颤被归类为特发性。针对急性缺血有很好的预防和治疗方法，对于预激综合征也有治愈性治疗。特发性室颤患者无法预先识别。然而，Brugada综合征和长QT综合征存在心电图和基因标志物。因此，对于这两种尚无有效治疗方法的综合征，讨论预防性除颤器预防猝死的可能作用是合理的。Brugada综合征患者猝死发生率高，对于电生理检查可诱发心律失常的患者，无论有无症状，均建议植入预防性除颤器。相反，长QT综合征的猝死发生率很低，植入预防性除颤器不具有成本效益。
