JAMA:肺动脉高压(PAH)最新定义、病理生理学和患病率

2022-05-24 刘少飞 MedSci原创

PAH是肺动脉高压(PH)的一种亚型,以肺动脉重构为特征。在美国,PAH 的患病率约为每 100 万成年人 10.6 例。未经治疗,PAH 会发展为右心衰竭和死亡。

肺动脉高压 (PH) 的临床分类,其中 PAH 是一种亚型,对于了解 PAH 的诊断和治疗方法很重要。目前,肺动脉高压的定义是在静息状态下,在仰卧位右心导管插入术中平均肺动脉压大于20 mmHg。该定义不同于之前 25 mmHg 或更高的阈值,因为与平均肺动脉压为20 mmHg或更低。

PAH的病理生理学

区分由于肺前毛细血管异常和肺后毛细血管异常引起的 PH 对于了解 PAH 的病理生理学至关重要(图1)。

肺动脉高压的定义是肺动脉内的压力升高。正常情况下,右心室的心输出量流经肺动脉和小动脉、肺毛细血管、肺小静脉和静脉,最终到达左心房。当肺动脉压力升高是由于肺毛细血管近端肺动脉阻力增加而导致肺动脉压力升高时存在毛细血管前 PH,并且始终存在于 PAH 中。

PAH的病理生理机制仍不清楚。血管病理学包括动脉内膜和内膜重塑、丛状病变和弹力层碎裂(图 2)。

血管重塑,定义为通过肥大或增生使血管壁增厚,主要导致肺血管阻力增加,随后平均肺动脉压增加。血管收缩是 PAH 的重要组成部分;然而,究竟是诱发事件还是肺血管重构的并发症尚不确定。最终,小毛细血管前动脉和小动脉被闭塞,导致 PAH 患者的一氧化碳 (DLCO) 弥散能力单独降低。有效的治疗理想地针对肺动脉的重塑以及血管收缩。最初,右心室通过增加收缩性和壁厚来补偿增加的后负荷。最终,右心室扩张并衰竭,如果不及时治疗,会因右心衰竭而死亡。

PAH 的患病率和患者特征

肺动脉高压并不常见。法国登记处(n = 674;2002-2003)报告每年每 100 万人中有 2.4 例发病率,每 100 万成年人中有 15 例患病率。位于美国的 REVEAL 登记(n = 2525;2006-2007)是一项涉及 54 个中心的多中心前瞻性队列研究,报告的发病率为每年每 100 万人 2.0 例,患病率为每 100 万成人 10.6 例。患者特征因登记而异。与 1981 年开始的美国国立卫生研究院登记 (n = 187) 的结果相反,REVEAL 登记 (n = 2525; 2006-2007) 报告说 PAH 患者年龄较大(年龄 50.1 [SD, 14.4] 岁 vs诊断时 35 [SD, 15] 年)并且在女性中占优势(女性与男性患者的比例,4.07:1 vs 1.7:1)。确定这些差异是否代表 PAH 患者特征的时间变化或与意识增强有关。在开发 PAH 特异性疗法之前,诊断后的 5 年生存率为 34%(n = 187)。最近的数据报告 1 年生存率约为 61%(REVEAL 登记;n = 2749;2006-2009)和 64%(法国登记;n = 1611;2006-2018)。

参考文献:

Ruopp NF, Cockrill BA. Diagnosis and Treatment of Pulmonary Arterial Hypertension: A Review. JAMA. 2022;327(14):1379–1391. doi:10.1001/jama.2022.4402

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    2022-06-29 贵阳
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    2022-10-18 hukaixun
  4. 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createdName=贵阳, createdTime=Wed Jun 29 12:22:50 CST 2022, time=2022-06-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1949338, encodeId=8728194933847, content=<a href='/topic/show?id=5ed95833172' target=_blank style='color:#2F92EE;'>#新定义#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=58331, encryptionId=5ed95833172, topicName=新定义)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=6ab940, createdName=hukaixun, createdTime=Tue Oct 18 19:22:50 CST 2022, time=2022-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1980992, encodeId=77cd19809928b, content=<a href='/topic/show?id=851de10460f' target=_blank style='color:#2F92EE;'>#病理生理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=52, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71046, encryptionId=851de10460f, topicName=病理生理学)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ba5f232, createdName=zhao4616, createdTime=Thu Dec 22 03:22:50 CST 2022, time=2022-12-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1701798, encodeId=bc121e01798c6, content=<a href='/topic/show?id=9c35e104573' target=_blank style='color:#2F92EE;'>#病理生理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71045, encryptionId=9c35e104573, topicName=病理生理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d59730392519, createdName=许安, createdTime=Sun Jan 29 19:22:50 CST 2023, time=2023-01-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1222089, encodeId=432c122208915, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=1, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a3845482238, createdName=泡泡糖111, createdTime=Tue May 24 14:54:58 CST 2022, time=2022-05-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1222092, encodeId=578b1222092f0, content=学习中, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=1, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=21ed8106057, createdName=梅斯徐, createdTime=Tue May 24 15:07:05 CST 2022, time=2022-05-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1752827, encodeId=59b21e528272a, content=<a href='/topic/show?id=09736951388' target=_blank style='color:#2F92EE;'>#生理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=69513, encryptionId=09736951388, topicName=生理学)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=1b3136571640, createdName=chenzhuanggui, createdTime=Tue Nov 15 01:22:50 CST 2022, time=2022-11-15, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1250817, encodeId=e58d125081e34, content=<a href='/topic/show?id=7d6f52988e1' target=_blank style='color:#2F92EE;'>#患病率#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=52988, encryptionId=7d6f52988e1, topicName=患病率)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f49517, createdName=xinmeili, createdTime=Wed May 25 16:22:50 CST 2022, time=2022-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1460204, encodeId=99ff146020466, content=<a href='/topic/show?id=1519136e641' target=_blank style='color:#2F92EE;'>#PAH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13676, encryptionId=1519136e641, topicName=PAH)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=87966261195, createdName=hb2010ye, createdTime=Wed May 25 16:22:50 CST 2022, time=2022-05-25, status=1, ipAttribution=)]
  5. [GetPortalCommentsPageByObjectIdResponse(id=1925602, encodeId=a58319256021e, content=<a href='/topic/show?id=26d633354c9' target=_blank style='color:#2F92EE;'>#动脉高压#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=51, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=33354, encryptionId=26d633354c9, topicName=动脉高压)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=44a360, createdName=jklm09, createdTime=Tue Oct 11 10:22:50 CST 2022, time=2022-10-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1736433, encodeId=136a1e36433ef, content=<a href='/topic/show?id=a421604e721' target=_blank style='color:#2F92EE;'>#最新#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=60477, encryptionId=a421604e721, topicName=最新)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4bd534165494, 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topicName=病理生理学)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ba5f232, createdName=zhao4616, createdTime=Thu Dec 22 03:22:50 CST 2022, time=2022-12-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1701798, encodeId=bc121e01798c6, content=<a href='/topic/show?id=9c35e104573' target=_blank style='color:#2F92EE;'>#病理生理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71045, encryptionId=9c35e104573, topicName=病理生理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d59730392519, createdName=许安, createdTime=Sun Jan 29 19:22:50 CST 2023, time=2023-01-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1222089, encodeId=432c122208915, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=1, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a3845482238, createdName=泡泡糖111, createdTime=Tue May 24 14:54:58 CST 2022, time=2022-05-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1222092, encodeId=578b1222092f0, content=学习中, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=1, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=21ed8106057, createdName=梅斯徐, createdTime=Tue May 24 15:07:05 CST 2022, time=2022-05-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1752827, encodeId=59b21e528272a, content=<a href='/topic/show?id=09736951388' target=_blank style='color:#2F92EE;'>#生理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=69513, encryptionId=09736951388, topicName=生理学)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=1b3136571640, createdName=chenzhuanggui, createdTime=Tue Nov 15 01:22:50 CST 2022, time=2022-11-15, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1250817, encodeId=e58d125081e34, content=<a href='/topic/show?id=7d6f52988e1' target=_blank style='color:#2F92EE;'>#患病率#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=52988, encryptionId=7d6f52988e1, topicName=患病率)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f49517, createdName=xinmeili, createdTime=Wed May 25 16:22:50 CST 2022, time=2022-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1460204, encodeId=99ff146020466, content=<a href='/topic/show?id=1519136e641' target=_blank style='color:#2F92EE;'>#PAH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13676, encryptionId=1519136e641, topicName=PAH)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=87966261195, createdName=hb2010ye, createdTime=Wed May 25 16:22:50 CST 2022, time=2022-05-25, status=1, ipAttribution=)]
  6. [GetPortalCommentsPageByObjectIdResponse(id=1925602, encodeId=a58319256021e, content=<a href='/topic/show?id=26d633354c9' target=_blank style='color:#2F92EE;'>#动脉高压#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=51, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=33354, encryptionId=26d633354c9, topicName=动脉高压)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=44a360, createdName=jklm09, createdTime=Tue Oct 11 10:22:50 CST 2022, time=2022-10-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1736433, encodeId=136a1e36433ef, content=<a href='/topic/show?id=a421604e721' target=_blank style='color:#2F92EE;'>#最新#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=60477, encryptionId=a421604e721, topicName=最新)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4bd534165494, createdName=贵阳, createdTime=Wed Jun 29 12:22:50 CST 2022, time=2022-06-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1949338, encodeId=8728194933847, content=<a href='/topic/show?id=5ed95833172' target=_blank style='color:#2F92EE;'>#新定义#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=58331, encryptionId=5ed95833172, topicName=新定义)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=6ab940, createdName=hukaixun, createdTime=Tue Oct 18 19:22:50 CST 2022, time=2022-10-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1980992, encodeId=77cd19809928b, content=<a href='/topic/show?id=851de10460f' target=_blank style='color:#2F92EE;'>#病理生理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=52, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71046, encryptionId=851de10460f, topicName=病理生理学)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ba5f232, createdName=zhao4616, createdTime=Thu Dec 22 03:22:50 CST 2022, time=2022-12-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1701798, encodeId=bc121e01798c6, content=<a href='/topic/show?id=9c35e104573' target=_blank style='color:#2F92EE;'>#病理生理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71045, encryptionId=9c35e104573, topicName=病理生理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d59730392519, createdName=许安, createdTime=Sun Jan 29 19:22:50 CST 2023, time=2023-01-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1222089, encodeId=432c122208915, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=1, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a3845482238, createdName=泡泡糖111, createdTime=Tue May 24 14:54:58 CST 2022, time=2022-05-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1222092, encodeId=578b1222092f0, content=学习中, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=1, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=21ed8106057, createdName=梅斯徐, createdTime=Tue May 24 15:07:05 CST 2022, time=2022-05-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1752827, encodeId=59b21e528272a, content=<a href='/topic/show?id=09736951388' target=_blank style='color:#2F92EE;'>#生理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=69513, encryptionId=09736951388, topicName=生理学)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=1b3136571640, createdName=chenzhuanggui, createdTime=Tue Nov 15 01:22:50 CST 2022, time=2022-11-15, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1250817, encodeId=e58d125081e34, content=<a href='/topic/show?id=7d6f52988e1' target=_blank style='color:#2F92EE;'>#患病率#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=52988, encryptionId=7d6f52988e1, topicName=患病率)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f49517, createdName=xinmeili, createdTime=Wed May 25 16:22:50 CST 2022, time=2022-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1460204, encodeId=99ff146020466, content=<a href='/topic/show?id=1519136e641' target=_blank style='color:#2F92EE;'>#PAH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13676, encryptionId=1519136e641, topicName=PAH)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=87966261195, createdName=hb2010ye, createdTime=Wed May 25 16:22:50 CST 2022, time=2022-05-25, status=1, ipAttribution=)]
    2022-05-24 泡泡糖111

    学习了

    1

    展开1条回复
  7. [GetPortalCommentsPageByObjectIdResponse(id=1925602, encodeId=a58319256021e, content=<a href='/topic/show?id=26d633354c9' target=_blank style='color:#2F92EE;'>#动脉高压#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=51, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=33354, encryptionId=26d633354c9, topicName=动脉高压)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=44a360, createdName=jklm09, createdTime=Tue Oct 11 10:22:50 CST 2022, time=2022-10-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1736433, encodeId=136a1e36433ef, content=<a href='/topic/show?id=a421604e721' target=_blank style='color:#2F92EE;'>#最新#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=60477, encryptionId=a421604e721, topicName=最新)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4bd534165494, 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topicName=病理生理学)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=ba5f232, createdName=zhao4616, createdTime=Thu Dec 22 03:22:50 CST 2022, time=2022-12-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1701798, encodeId=bc121e01798c6, content=<a href='/topic/show?id=9c35e104573' target=_blank style='color:#2F92EE;'>#病理生理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71045, encryptionId=9c35e104573, topicName=病理生理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d59730392519, createdName=许安, createdTime=Sun Jan 29 19:22:50 CST 2023, time=2023-01-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1222089, encodeId=432c122208915, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=1, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=a3845482238, createdName=泡泡糖111, createdTime=Tue May 24 14:54:58 CST 2022, time=2022-05-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1222092, encodeId=578b1222092f0, content=学习中, beContent=null, objectType=article, channel=null, level=null, likeNumber=62, replyNumber=1, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=21ed8106057, createdName=梅斯徐, createdTime=Tue May 24 15:07:05 CST 2022, time=2022-05-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1752827, encodeId=59b21e528272a, content=<a href='/topic/show?id=09736951388' target=_blank style='color:#2F92EE;'>#生理学#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=69513, encryptionId=09736951388, topicName=生理学)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=1b3136571640, createdName=chenzhuanggui, createdTime=Tue Nov 15 01:22:50 CST 2022, time=2022-11-15, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1250817, encodeId=e58d125081e34, content=<a href='/topic/show?id=7d6f52988e1' target=_blank style='color:#2F92EE;'>#患病率#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=46, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=52988, encryptionId=7d6f52988e1, topicName=患病率)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=f49517, createdName=xinmeili, createdTime=Wed May 25 16:22:50 CST 2022, time=2022-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1460204, encodeId=99ff146020466, content=<a href='/topic/show?id=1519136e641' target=_blank style='color:#2F92EE;'>#PAH#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=13676, encryptionId=1519136e641, topicName=PAH)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=87966261195, createdName=hb2010ye, createdTime=Wed May 25 16:22:50 CST 2022, time=2022-05-25, status=1, ipAttribution=)]
    2022-05-24 梅斯徐

    学习中

    1

    展开1条回复
  8. 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    2022-05-25 hb2010ye

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