Brugada综合征,千万别搞错!

2018-09-18 人世间 医学界心血管频道

各位看官作出Brugada综合征这一诊断前,有排除过哪些疾病呢?

Brugada综合征以其经典的心电图特点为大家所熟知,其典型的心电图表现,又称为Brugada波,主要是指类右束支阻滞、V1-V3导联ST段下斜型或马鞍型抬高、及T波双向或倒置,其中根据心电图不同特征,又可分为3型,可见表1和图1。
  
表1:Brugada波分型



图1:Brugada波分型

值得注意的是,只有I型Brugada波才具有诊断价值,但是II型或III型Brugada波在改变胸前导联电极位置或药物激发时也可以转变为I型Brugada波。
 
另外,即使是I型Brugada波,也并不等同于Brugada综合征。所谓Brugada综合征,需满足“1+1/5”的诊断标准:“1”是指I型Brugada波,“1/5”是满足以下5条标准中的任意一条或更多,见表2。
 
表2:Brugada综合征诊断标准中的5条临床状况



那么问题来了,
满足以上诊断标准的,就一定是Brugada综合征吗?

身为从医多年的老司机你怎么看?
不妨,先来看一个病例↓↓↓
 
病例介绍

患者78岁男性,主因“发作性晕厥1天”就诊,否认猝死家族史。

急查心电图可见V1-V3导联ST段呈下斜型抬高,并伴有T波倒置,符合I型Brugada波,见图2。结合晕厥发作,该患者完全满足上述的“1+1/5”的诊断标准,Brugada综合征的诊断似乎呼之欲出。
  

图2:入院心电图示I型Brugada波
 
但是该患者发病年龄较大,同时仔细阅读上述心电图(图2),似乎也有点SIQIIITIII的意思。随后完善了一系列常规检查,发现D-Dimer升高伴有低氧血症,及超声心动示右心增大、肺动脉高压,而肺动脉CT也证实了肺栓塞的诊断。
 
也许有人会问,他有没有可能是肺栓塞合并Brugada综合征呢?

这个问题一般情况下很难回答,幸运的是该患者在行Holter检查中再次出现晕厥,而Holter并未记录到恶性心律失常,而这也更不可以排除Brugada综合征这一诊断。因此,该患者最终考虑肺栓塞。

肺栓塞为什么会出现Brugada波?

我想大家都很好奇,肺栓塞明明是肺血管病变,为什么会出现心脏离子通道疾病常见的Brugada波?

这可能与肺栓塞导致肺动脉高压,引起右室压力负荷增加或缺血,可激活ATP敏感性钾通道,使钾外流增加,减少或抑制了钙内流,同时原本就较强的瞬时外向钾电流(Ito)变得更强,从而表现出Brugada波。
 
还有那些疾病可出现Brugada波?

除了肺栓塞以外,哪些疾病也可以表现为Brugada波呢?我想这也是大家极其在意的问题。

有学者将非先天性因素引起而是后天获得性临床疾病所导致的Brugada波称为Brugada拟表型,并根据病因分为六类:

代谢状况;
机械压迫;
缺血和肺栓塞; 
心肌和心包疾病;
心电图调制(基于不恰当的高通滤波);
其他,如酗酒、发热等

而这些是我们需在诊断Brugada综合征前需要慎重排除的。

对于Brugada综合征,我们很多时候是需要考虑植入式心脏转复除颤器(ICD)植入的,而对于Brugada拟表型,其临床过程多数良性,去除病因后Brugada波能被逆转,预后良好。所以希望各位看官在以后作出Brugada综合征这一诊断前,能够想起这一小短文。
 
原始出处:

1.Dendramis G. Brugada syndrome and Brugada phenocopy. The importance of a differential diagnosis[J]. International Journal of Cardiology, 2016, 210:25-27.

2.Vohra J, Rajagopalan S, Group CGCW: Update on the Diagnosis and Management of Brugada Syndrome. Heart, lung & circulation 2015, 24(12):1141-1148.

3.张妮潇: 急性肺栓塞伴晕厥患者心电图Brugada波1例. 临床心电学杂志 2017, 1(26).

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    2019-04-27 小豁牙

    学习了,有收获,收藏起来!

    0

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    2018-09-20 般若傻瓜
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    2018-09-20 nymo
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    2018-09-19 123d926dm06暂无昵称

    快乐学习每一天

    0

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    2018-09-18 医者仁心5538

    学习了

    0

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患有Brugada综合征的年轻患者发生室性心律失常的风险很高,植入型心律转复除颤器(ICD)可以较好地预防这种风险,然而这种治疗方法可能会引起植入设备相关并发症和误放电。本研究的目的旨在评估植入有ICD的Brugada综合征年轻患者的临床特征、管理以及预后。本研究纳入了35例年龄≤20岁的植入ICD的Brugada综合征患者,植入ICD的平均年龄为13.9 ± 6.2岁,92%的患者有较明显的临床

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猝死恐慌曾笼罩东南亚上世纪数十年间,一个可怕的谜团如幽灵般游荡在东南亚各国,从菲律宾到泰国,从缅甸到老挝,众多平素健康的青壮年在夜间不明原因地猝死,而且绝大多数为男性。统计数据表明,青壮年发生这种神秘死亡的人数位列交通事故死亡人数之后,高居第二位,且猝死还呈家族聚集性,从而造成了更大程度的恐慌。为何死神如此无情又频频光顾,无人能够回答,医学也无法给予合理的解释。  当科学被阴霾

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