NEJM:活久见! 碰上一个ST段压低36年的“没事人”

2018-11-15 泰中 医学界心血管频道

心肌缺血的心电图表现会持续数十年吗?

近期,《新英格兰医学杂志(NEJM)》上刊登了一则病例,引起了笔者的关注,一位才60多岁的患者,竟已和“ST段压低”安然生活了36年,调查后发现这背后远不是个例那么简单,一起来看看吧~

病例

■ 现病史:63岁老年男性,自诉心电图有恙持续36年,心电图提示广泛ST段凹面向上压低(见图1),无其他异常症状。

■ 既往史:患者55岁时出现阵发性房颤,予氟卡胺药物复律治疗。无QTc延长或Brugada综合征表型。住院期间曾经发生过一次11秒的呼吸停止,曾植入心房、心室双腔起搏器。停用氟卡胺后,患者曾行房颤导管消融治疗。


图1:I,II,aVL,aVF和V2-V6导联均出现ST段凹面向上压低

■ 实验室检查:电解质、肾功能、肝功能和甲状腺功能均正常,询问病史前未服药,重复负荷试验显示血压和心率变化正常,只在体力活动时出现ST段压低加深。

47岁时患者曾行超声心动图检查,结果正常;
48岁和63岁时曾行冠脉造影,结果同样正常;
62岁时超声心动图显示左室射血分数45%-50%,心房扩张;
Holter监测显示房颤心律,24小时内2500次室性早搏。

这则病例中最令人疑惑的是,这位患者持续了30年的无症状心电图异常究竟是什么?

从心电图入手,暗藏玄机?

从心电图上分析,患者为窦性心律,P波低电压,PR和QT间期正常,QRS形状时限均正常,但在I、II、aVL、aVF以及V2-V6导联均出现ST段凹面向上压低。在ST段和T波间无明显转折,T波上升段可见一切迹,在V1导联可见ST段升高。

那么,为何会出现大面积的ST段压低呢?

■ 心肌缺血? 

在缺血性心脏病的心电图中,ST段压低常对应心内膜损伤。不同导联可记录到不同位置的心内膜损伤情况,其定位表现则取决于冠脉部位和损伤区域。因此,ST段压低心电图的部分波形能给予一定的提示。

比如说当胸前导联广泛出现ST段压低,尤其以V4-V6导联显著时,提示可能是前降支次全闭塞。但患者心电图持续三十年稳定没有变化,且病史和冠脉造影均不支持心肌缺血的诊断

■ Brugada综合征?

Brugada综合征作为非结构性心脏病患者发生心源性猝死的主要病因之一,这类患者通常都有晕厥和致死性心律失常的病史。为了明确诊断Brugada综合征需要在至少一个右侧胸前导联(V1~V3)出现Brugada模式心电图改变和相应临床表现(如房颤,多形性室速等室性心律失常)。

该患者出现了V1-V3导联的ST段改变,房颤和室性快速性心律失常,但是广泛ST段压低不属于Brugada模式心电图改变(V1~V3导联出现J波+ST段穹窿样抬高和末段下斜+T波倒置);


图2:典型的Brugada模式心电图

不是个例,一家子ST段都低!

原来如此特异的心电图竟然不是个例,患者的儿子和女儿身上也存在相似的心电图改变!扩大调查发现,家系里还有几位成员心电图也出现这种稳定的ST段压低。这家人里有2位亲戚发生了心源性猝死,3位在50岁的时候罹患房颤,而他们都未被诊断过冠脉相关疾病。

根据调查结果,绘制了患者的家系图(图3),他的哥哥、侄女、侄孙、儿子、女儿和孙子都出现了表型,俨然是一个常染色体显性遗传病。


图3:患者的家系图,P为先证者

知识点!家族性ST段压低综合征

来自丹麦、荷兰、英国的多个中心共采集到5个家系,都出现了类似的特征:心电图改变表现为持续、稳定、显著的非缺血性ST段压低,缓慢发生房颤以及室性心律失常,伴随有时轻度的左室功能不全,呈常染色体显性遗传。

他们的ST段压低在儿童期即出现,稳定持续,在体力活动时压低会加深。除此以外可保持多年无任何症状,但就像本文主角一样,在30岁到50岁间会逐渐发生房性心律失常,50岁时发生室性心律失常,伴随心功能下降。

专家们把这些家系罹患的疾病进行了归纳,提出一个新的遗传性心脏综合征——家族性ST段压低综合征,并提出了其诊断标准:

无法解释的超过7个导联上出现的J点后80ms处ST段凹面向上压低≥0.1mV。
aVR导联ST段抬高≥0.1mV。
无正常心电图表现,心电图异常持续(可因体力活动加重)。
常染色体显性遗传。


虽然这种疾病早期几乎无任何异常症状,但却是实打实的遗传性要命综合征啊!随年龄增加,几乎全部会发展成以房颤为代表的房性心律失常,甚至会出现突发性室性快速性心律失常,发生猝死!

因此,早期识别诊断,提前建议患者和家系筛查、治疗,可预防一个家族的不幸!

原始出处:

Bundgaard H, J?ns C, et al.  A Novel Familial Cardiac Arrhythmia Syndrome with Widespread ST-Segment Depression. N Engl J Med. 2018 Nov 1;379(18):1780-1781. doi: 10.1056/NEJMc1807668. 

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    2018-12-18 wxl882001

    了解一下

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=355753, encodeId=0c25355e5304, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=55, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Tue Dec 18 18:16:32 CST 2018, time=2018-12-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1624400, encodeId=3e601624400e3, content=<a href='/topic/show?id=f27b16862d9' target=_blank style='color:#2F92EE;'>#ST段#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=16862, encryptionId=f27b16862d9, topicName=ST段)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=676820671866, createdName=hyf035, createdTime=Sat Nov 17 02:03:00 CST 2018, time=2018-11-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352730, encodeId=cfea352e308d, content=学习学习学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20231215/ac08b4d4cecf4bd3b9af86e00dcbca38/f6b6a60efd854ff191eb5c611ec7dd12.jpg, createdBy=8afb2071832, createdName=15938038573红旗飘飘, createdTime=Fri Nov 16 06:25:57 CST 2018, time=2018-11-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352724, encodeId=fd43352e24b7, content=长见识了, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=6d7d2445101, createdName=花千树, createdTime=Fri Nov 16 02:08:54 CST 2018, time=2018-11-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352688, encodeId=2e33352688b0, content=好病例, beContent=null, objectType=article, channel=null, level=null, likeNumber=65, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=c4a5105539, createdName=lovetcm, createdTime=Thu Nov 15 20:05:42 CST 2018, time=2018-11-15, status=1, ipAttribution=)]
    2018-11-17 hyf035
  3. [GetPortalCommentsPageByObjectIdResponse(id=355753, encodeId=0c25355e5304, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=55, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Tue Dec 18 18:16:32 CST 2018, time=2018-12-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1624400, encodeId=3e601624400e3, content=<a href='/topic/show?id=f27b16862d9' target=_blank style='color:#2F92EE;'>#ST段#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=16862, encryptionId=f27b16862d9, topicName=ST段)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=676820671866, createdName=hyf035, createdTime=Sat Nov 17 02:03:00 CST 2018, time=2018-11-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352730, encodeId=cfea352e308d, content=学习学习学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20231215/ac08b4d4cecf4bd3b9af86e00dcbca38/f6b6a60efd854ff191eb5c611ec7dd12.jpg, createdBy=8afb2071832, createdName=15938038573红旗飘飘, createdTime=Fri Nov 16 06:25:57 CST 2018, time=2018-11-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352724, encodeId=fd43352e24b7, content=长见识了, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=6d7d2445101, createdName=花千树, createdTime=Fri Nov 16 02:08:54 CST 2018, time=2018-11-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352688, encodeId=2e33352688b0, content=好病例, beContent=null, objectType=article, channel=null, level=null, likeNumber=65, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=c4a5105539, createdName=lovetcm, createdTime=Thu Nov 15 20:05:42 CST 2018, time=2018-11-15, status=1, ipAttribution=)]
    2018-11-16 15938038573红旗飘飘

    学习学习学习了

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=355753, encodeId=0c25355e5304, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=55, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Tue Dec 18 18:16:32 CST 2018, time=2018-12-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1624400, encodeId=3e601624400e3, content=<a href='/topic/show?id=f27b16862d9' target=_blank style='color:#2F92EE;'>#ST段#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=16862, encryptionId=f27b16862d9, topicName=ST段)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=676820671866, createdName=hyf035, createdTime=Sat Nov 17 02:03:00 CST 2018, time=2018-11-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352730, encodeId=cfea352e308d, content=学习学习学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20231215/ac08b4d4cecf4bd3b9af86e00dcbca38/f6b6a60efd854ff191eb5c611ec7dd12.jpg, createdBy=8afb2071832, createdName=15938038573红旗飘飘, createdTime=Fri Nov 16 06:25:57 CST 2018, time=2018-11-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352724, encodeId=fd43352e24b7, content=长见识了, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=6d7d2445101, createdName=花千树, createdTime=Fri Nov 16 02:08:54 CST 2018, time=2018-11-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=352688, encodeId=2e33352688b0, content=好病例, beContent=null, objectType=article, channel=null, level=null, likeNumber=65, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=c4a5105539, createdName=lovetcm, createdTime=Thu Nov 15 20:05:42 CST 2018, time=2018-11-15, status=1, ipAttribution=)]
    2018-11-16 花千树

    长见识了

    0

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    2018-11-15 lovetcm

    好病例

    0

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骑白马的不一定是唐僧,还可能是我们心目中万分期待的王子。因此,当胸痛+ST段抬高+肌钙蛋白(cTn)升高找上门时,其背后的元凶也许并不十分可怕,而是急性心梗的“最善意的模仿者”。

ST段呈动态演变的de Winter综合征1例

患者男性,43岁,因"突发胸痛6 h"于2017年11月23日入院。患者入院6 h前劳累后突感胸痛,持续不可缓解,由急救车送入我院急诊,急救车中行心电图示Ⅱ、aVF、V2~V6导联J点下移,伴ST段上斜型压低,aVR导联ST段轻度抬高(图1)。