[SCD-FORUM] 39E:无症状性Brugada综合征患者 Zhang Li医生

[SCD Symposium]

39E:无症状性Brugada综合征患者    Zhang Li医生 这样一 
来，我们需要自问一下为何这样，Ramon Brugada医生。 
在未明确疾病本质的情况下，我们可能不能进行危险 
度分层。如果Brugada综合征是一种原发性复极易常， 
则在室速/室颤发作前应该出现复极不稳定如T波交替 
（mv）。事实上，大半室速/室颤患者显示出正后电位 
( Arch Cardiol Mex. 2006 Jan-Mar;76(1):52-8 )。近期Shimizu医生 
的小组证实除极化和复极化均有异常[ Pacing Clin  
Electrophysiol.2006 Oct;29(10):1112-21]。过去，我们主要关注 
复极1期右心室心外膜钠电流的减少以及复极时透壁 
传播的结果，这可能是部分机理。但不应忽视由于钠 
离子流本身减少导致了右室流出道（ROVT）区域兴奋 
传播变慢。（Am J Physiol Heart Circ Physiol. 2006 Jul 28; [Epub  
ahead of print]）尽管常规检查无法察觉，但通道疾病尤 
其是SCN5A基因突变能导致结构异常。这些结构的变化 
（宏观或微观）也能导致折返性室性快速性心律失常 
和室速/室颤性猝死。
                                                                         
    致


Li Zhang, MDLDS 医院,Utah大学医学院Salt Lake City, UT
USA

潘佳君译 王玲洁校


39E RE: Asymptomatic patient with Brugada syndrome. Dr. Li Zhang

Then we should ask oursselves why is so ，Dr.Ramon Brugada. Without  
a  clear understanding of the disease nature, we may be always in the  
dark in terms of risk stratification. If Brugada syndrome were a  
primary repolarization disorder, one would expect the repolarization  
instability such as microvolt T wave alternans prior to the onset of   
VT/VF. As a matter of fact, the majority of VT/VF patients showed  
positive late potentials ( Arch Cardiol Mex. 2006 Jan-Mar;76(1): 
52-8 ). Recently Dr. Shimizu's group demonstrated both depolarization  
and repolarization abnormalities [ Pacing Clin Electrophysiol. 2006   
Oct;29(10):1112-21].In the past our main focus was the consequence of  
reduced INa to the phase 1 repolarization in the RV epicardium and  
the transmural dispersion of repolarization, which may be part of the  
story. The slow activation propagation in ROVT region due to reduced  
sodium current itself should not be overlooked. Am J Physiol Heart   
Circ Physiol. 2006 Jul 28; [Epub ahead of print] Channelopathies  
especially SCN5A mutations can lead to structural abnormalities  
although many are undetectable by routine work up. Those structural  
alterations (macroscopic or microscopic) could also be the substrate  
to re-entrant ventricular tachyarrhythmias and sudden VT/VF  
death.Sincerely,Li Zhang

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee


>
> 31E 加拿大蒙特利尔大学的Ramon Brugada 博士回答 ：
>
> 据我所知，目前关于T波电交替与Brugada综合征关系的
> 相关研究有两项（日本和加拿大），但两项都未证实
> 二者之间有相关性。
> 答疑者简介：Ramon Brugada，医学博士，美国心脏病学
> 学会会员，副教授
> 加拿大心律失常遗传学研究学会主任研究员
> 加拿大蒙特利尔大学临床心血管病遗传中心主任
> 蒙特利尔心脏协会会员
> 通信地址：  5000 Rue Belanger
> Montreal, QC H1T 1C8
> Canada
> 邮箱：ramon at brugada.org
> Sergio Dubner         学术委员会主席
> Edgardo Schapachnik   指导委员会主席
>
> 董颖雪译 王玲洁校
>
> 31E RE: Asymptomatic patient with Brugada syndrome. Dr. Ramon Brugada
> No that i know of. There are two studies which looked at TWA (Japan  
> and
> Germany) in Brugada syndrome. Neither one showed any correlation with
> risk.
> Dr. Ramon Brugada MD FACC
> Associate Professor of Medicine
> Canadian Research Chair Genetics of Arrhythmias
> University of Montreal
> Director Clinical Cardiovascular Genetics Center
> Montreal Heart Institute
> 5000 Rue Belanger
> Montreal, QC H1T 1C8
> Canada
> ramon at brugada.org