心血管

订阅

MedSci主页 / 所有栏目 / 心血管

Circulation：转甲状腺素淀粉样变性心肌病——老年人的又一杀手

2019-07-06 MedSci MedSci原创

转甲状腺素淀粉样变性心肌病ATTR-CM心衰V122I
关注

转甲状腺素淀粉样变性心肌病(ATTR-CM)是老年人心力衰竭的一个病因。现研究人员对ATTR-CM的发病过程进行归纳总结，并比较获得性和遗传性ATTR-CM患者的预后和生活质量。研究人员共纳入711位野生型ATTR-CM患者，其中205位是携带V1221突变的遗传性ATTR-CM（V122I-hATTR-CM），另118位为非-V122I-hATTR-CM。前瞻性地评估患者的心指数、功能状态（6分

转甲状腺素淀粉样变性心肌病(ATTR-CM)是老年人心力衰竭的一个病因。现研究人员对ATTR-CM的发病过程进行归纳总结，并比较获得性和遗传性ATTR-CM患者的预后和生活质量。

研究人员共纳入711位野生型ATTR-CM患者，其中205位是携带V1221突变的遗传性ATTR-CM（V122I-hATTR-CM），另118位为非-V122I-hATTR-CM。前瞻性地评估患者的心指数、功能状态（6分钟步行试验）、生活质量和存活率。

确诊延迟严重，患者在确诊前3年内到医院就诊的中位次数为17次（9-27次），在这段时间内，患者的生活质量较差。有42%的患者在出现心脏症状后4年才确诊为野生型ATTR-CM。与其他亚组患者相比，V122I-hATTR-CM患者，在确诊时的心功能损伤更严重（p<0.001），相关指标更差（p<0.001），生活质量下降更明显，存活率也更低（p<0.001）。

ATTR-CM是一种不可逆转的、可导致死亡的进行性心肌病，患者生活质量严重下降。出现症状后几年才能明确诊断。提高对ATTR-CM的认知、更广泛地应用已获得验证的影像诊断方法或可改善患者预后。

原始出处：

Thirusha Lane， et al. Natural History， Quality of Life， and Outcome in Cardiac Transthyretin Amyloidosis. https：//doi.org/10.1161/CIRCULATIONAHA.118.038169Circulation. 2019；140：16–26

本文系梅斯医学（MedSci）原创编译，转载需授权！

版权声明：
本网站所有内容来源注明为“梅斯医学”或“MedSci原创”的文字、图片和音视频资料，版权均属于梅斯医学所有。非经授权，任何媒体、网站或个人不得转载，授权转载时须注明来源为“梅斯医学”。其它来源的文章系转载文章，或“梅斯号”自媒体发布的文章，仅系出于传递更多信息之目的，本站仅负责审核内容合规，其内容不代表本站立场，本站不负责内容的准确性和版权。如果存在侵权、或不希望被转载的媒体或个人可与我们联系，我们将立即进行删除处理。
在此留言
评论区 (5)
#插入话题
  1. [GetPortalCommentsPageByObjectIdResponse(id=1405171, encodeId=3afc14051e140, content=<a href='/topic/show?id=cb6e6591850' target=_blank style='color:#2F92EE;'>#淀粉样变#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65918, encryptionId=cb6e6591850, topicName=淀粉样变)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=2ca02527521, createdName=xjy03, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1434673, encodeId=951e14346e36a, content=<a href='/topic/show?id=940265920ab' target=_blank style='color:#2F92EE;'>#淀粉样变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65920, encryptionId=940265920ab, topicName=淀粉样变性)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463683, encodeId=7fac14636836d, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622395, encodeId=166116223952a, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=369189, encodeId=fcf236918987, content=666, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/01/04/2540fb84decb81d47a7f2868070e6cbd.jpg, createdBy=ed3f2205793, createdName=百草, createdTime=Sat Jul 06 23:11:10 CST 2019, time=2019-07-06, status=1, ipAttribution=)]
    2019-07-08 xjy03

    #淀粉样变#

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=1405171, encodeId=3afc14051e140, content=<a href='/topic/show?id=cb6e6591850' target=_blank style='color:#2F92EE;'>#淀粉样变#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65918, encryptionId=cb6e6591850, topicName=淀粉样变)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=2ca02527521, createdName=xjy03, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1434673, encodeId=951e14346e36a, content=<a href='/topic/show?id=940265920ab' target=_blank style='color:#2F92EE;'>#淀粉样变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65920, encryptionId=940265920ab, topicName=淀粉样变性)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463683, encodeId=7fac14636836d, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622395, encodeId=166116223952a, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=369189, encodeId=fcf236918987, content=666, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/01/04/2540fb84decb81d47a7f2868070e6cbd.jpg, createdBy=ed3f2205793, createdName=百草, createdTime=Sat Jul 06 23:11:10 CST 2019, time=2019-07-06, status=1, ipAttribution=)]
    2019-07-08 zhouqu_8

    #淀粉样变性#

    0

  3. [GetPortalCommentsPageByObjectIdResponse(id=1405171, encodeId=3afc14051e140, content=<a href='/topic/show?id=cb6e6591850' target=_blank style='color:#2F92EE;'>#淀粉样变#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65918, encryptionId=cb6e6591850, topicName=淀粉样变)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=2ca02527521, createdName=xjy03, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1434673, encodeId=951e14346e36a, content=<a href='/topic/show?id=940265920ab' target=_blank style='color:#2F92EE;'>#淀粉样变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65920, encryptionId=940265920ab, topicName=淀粉样变性)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463683, encodeId=7fac14636836d, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622395, encodeId=166116223952a, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=369189, encodeId=fcf236918987, content=666, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/01/04/2540fb84decb81d47a7f2868070e6cbd.jpg, createdBy=ed3f2205793, createdName=百草, createdTime=Sat Jul 06 23:11:10 CST 2019, time=2019-07-06, status=1, ipAttribution=)]
    2019-07-08 huangdf

    #变性#

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=1405171, encodeId=3afc14051e140, content=<a href='/topic/show?id=cb6e6591850' target=_blank style='color:#2F92EE;'>#淀粉样变#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65918, encryptionId=cb6e6591850, topicName=淀粉样变)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=2ca02527521, createdName=xjy03, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1434673, encodeId=951e14346e36a, content=<a href='/topic/show?id=940265920ab' target=_blank style='color:#2F92EE;'>#淀粉样变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65920, encryptionId=940265920ab, topicName=淀粉样变性)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463683, encodeId=7fac14636836d, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622395, encodeId=166116223952a, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=369189, encodeId=fcf236918987, content=666, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/01/04/2540fb84decb81d47a7f2868070e6cbd.jpg, createdBy=ed3f2205793, createdName=百草, createdTime=Sat Jul 06 23:11:10 CST 2019, time=2019-07-06, status=1, ipAttribution=)]
    2019-07-08 爆笑小医

    #肌病#

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=1405171, encodeId=3afc14051e140, content=<a href='/topic/show?id=cb6e6591850' target=_blank style='color:#2F92EE;'>#淀粉样变#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65918, encryptionId=cb6e6591850, topicName=淀粉样变)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=2ca02527521, createdName=xjy03, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1434673, encodeId=951e14346e36a, content=<a href='/topic/show?id=940265920ab' target=_blank style='color:#2F92EE;'>#淀粉样变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=65920, encryptionId=940265920ab, topicName=淀粉样变性)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1463683, encodeId=7fac14636836d, content=<a href='/topic/show?id=7d27381288e' target=_blank style='color:#2F92EE;'>#变性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=28, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=38128, encryptionId=7d27381288e, topicName=变性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f69f6545041, createdName=huangdf, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622395, encodeId=166116223952a, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Mon Jul 08 11:24:00 CST 2019, time=2019-07-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=369189, encodeId=fcf236918987, content=666, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2019/01/04/2540fb84decb81d47a7f2868070e6cbd.jpg, createdBy=ed3f2205793, createdName=百草, createdTime=Sat Jul 06 23:11:10 CST 2019, time=2019-07-06, status=1, ipAttribution=)]
    2019-07-06 百草

    666

    0