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[布加综合征——一个全新病例]

[Brugada syndrome--a brand new case].

作者信息

Jurcević Ruzica, Angelkov Lazar, Vukajlović Dejan, Ristić Velibor, Tomović Milosav, Kojić Dejan, Dukanović Bosko

机构信息

Institut za kardiovaskularne bolesti Dedinje, Odeljenje za elektrofiziologiju i elektrostimulaciju, Beograd, Srbija.

出版信息

Vojnosanit Pregl. 2009 Aug;66(8):667-70. doi: 10.2298/vsp0908667j.

DOI:10.2298/vsp0908667j
PMID:19780424
Abstract

BACKGROUND

Brugada syndrome (BS) is a disorder characterized by syncope or sudden death associated with one of several electrocardiographic (ECG) patterns characterized by incomplete right bundle branch block and ST elevation in the anterior precordial leads. Patients with BS are prone to develop ventricular tachyarrhythmias that may lead to syncope, cardiac arrest, or sudden cardiac death.

CASE REPORT

A 58-year-old woman is the first described case of Brugada syndrome in Serbia with intermittent typical changes in basic electrocardiography (ECG): ST segment elevation in the precordial chest leads like dome or coved--major form or type I. For the last 27 years the patient had suffered of palpitations and dizziness, without syncopal events. Her sister had died suddenly during the night in sleep. During 24-hour Holter monitoring the patient had ventricular premature beats during the night with R/T phenomenon and during the recovery phase of exercise testing had rare premature ventricular beats as the consequence of parasympatethic stimulation. Late potentials were positive. Echocardiography revealed left ventricular ejection fraction of 60%. We performed coronary angiography and epicardial coronary arteries were without significant stenosis and structural heart disease was excluded. In the bigining of the electrophysiological study ECG was normal, and after administration of Propaphenon i.v. Brugada syndrome unmasked with appearance of type I ECG pattern. A programed ventricular stimulation induced non sustained ventricular tachycardia. One-chamber implantable cardioverter defibrillator was implanted and the patient was treated with a combination od amiodarone and metoprolol per os. After one-year folow-up, there were no episodes of ventricular tachycardia and ventricular fibrillation.

CONCLUSION

Brugada syndrome is a myocardial disorder which prognosis and therapy are related to presence of ventricular fibrillation or ventricular tachycardia. Electrophysiologicaly induced malignant ventricular disorders class I are indication for implantation of cardioverter defibrilator, as also occurred in presented patient.

摘要

背景

Brugada综合征(BS)是一种以晕厥或猝死为特征的疾病,与几种心电图(ECG)模式之一相关,其特征为不完全性右束支传导阻滞和胸前导联ST段抬高。BS患者容易发生室性快速性心律失常,可能导致晕厥、心脏骤停或心源性猝死。

病例报告

一名58岁女性是塞尔维亚首例被描述的Brugada综合征病例,基础心电图(ECG)有间歇性典型变化:胸前导联ST段抬高呈穹窿形或下斜形——主要形式或I型。在过去27年里,该患者有心悸和头晕症状,但无晕厥事件。她的姐姐在夜间睡眠中突然死亡。在24小时动态心电图监测期间,患者夜间出现室性早搏伴R/T现象,运动试验恢复阶段因迷走神经刺激出现罕见的室性早搏。晚电位阳性。超声心动图显示左心室射血分数为60%。我们进行了冠状动脉造影,心外膜冠状动脉无明显狭窄,排除了结构性心脏病。在电生理研究开始时,心电图正常,静脉注射普罗帕酮后,Brugada综合征显现,出现I型心电图模式。程控心室刺激诱发非持续性室性心动过速。植入了单腔植入式心脏复律除颤器,患者口服胺碘酮和美托洛尔联合治疗。经过一年的随访,未出现室性心动过速和心室颤动发作。

结论

Brugada综合征是一种心肌疾病,其预后和治疗与心室颤动或室性心动过速的存在有关。电生理诱发的I类恶性室性疾病是植入心脏复律除颤器的指征,本病例患者也是如此。

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