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[Brugada综合征的近期临床情况]

[Recent clinical picture of Brugada syndrome].

作者信息

Dabek Józefa, Lepich Tomasz, Piechota Mieczysław

机构信息

Katedra i Klinika Kardiologii, Slaski Uniwersytet Medyczny w Katowicach.

出版信息

Pol Merkur Lekarski. 2011 Dec;31(186):364-7.

Abstract

Brugada syndrome is a genetically-based autosomal dominant condition. It occurs as a result of some disorder of ion channels which regulate currents responsible for development of activity potential. Brugada syndrome may appear as a family disease or as a sporadic condition. The syndrome can develop at every age of human life, yet, most frequently in the 3-4th life decade, mostly in men (85%). Its main symptoms include: malignant ventricular arrhythmia leading to fainting, and consequently accounting for, at least 4-12% of all sudden cardiac deaths and for 20-50% of deaths without any confirmed organic heart disease. The purpose of the paper was to present the currently-possessed knowledge about the condition and to highlight the necessity of an indepth ECG analysis, especially with respect to discovery of rare conditions. Due to different clinical symptoms of Brugada syndrome and a possibility of occurrence of changes in the ECG record, it is necessary to regularly monitor and check Brugada syndrome patients. Moreover, it is important to make both patients and first-contact doctors aware of a Brugada syndrome diagnosis and to inform them about a necessity of eliminating certain medicaments and factors (infections, intense physical activity) responsible for sudden cardiac death. Despite possessing great and advanced knowledge about the disorder, the implementation of cardioverter-defibrillator remains the only effective way of treating malignant arrhythmia and of preventing sudden cardiac death.

摘要

布加综合征是一种基于遗传的常染色体显性疾病。它是由调节动作电位形成电流的离子通道紊乱引起的。布加综合征可能以家族性疾病或散发性疾病的形式出现。该综合征可在人生的任何年龄段发病,但最常见于30至40岁,男性居多(85%)。其主要症状包括:导致昏厥的恶性室性心律失常,因此至少占所有心源性猝死的4%至12%,以及占无确诊器质性心脏病死亡病例的20%至50%。本文的目的是介绍目前关于该疾病的已有知识,并强调深入进行心电图分析的必要性,特别是对于发现罕见情况而言。由于布加综合征的临床症状各异,且心电图记录可能出现变化,因此有必要定期监测和检查布加综合征患者。此外,让患者和首诊医生了解布加综合征的诊断情况,并告知他们消除某些导致心源性猝死的药物和因素(感染、剧烈体育活动)的必要性也很重要。尽管对该疾病已有丰富和先进的认识,但植入心脏复律除颤器仍然是治疗恶性心律失常和预防心源性猝死的唯一有效方法。

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