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慢性血栓栓塞性肺动脉高压和慢性血栓栓塞性肺疾病

黄伸伸 邱家勇 毛毅敏

黄伸伸, 邱家勇, 毛毅敏. 慢性血栓栓塞性肺动脉高压和慢性血栓栓塞性肺疾病[J]. 罕见病研究, 2023, 2(1): 17-26. doi: 10.12376/j.issn.2097-0501.2023.01.003
引用本文: 黄伸伸, 邱家勇, 毛毅敏. 慢性血栓栓塞性肺动脉高压和慢性血栓栓塞性肺疾病[J]. 罕见病研究, 2023, 2(1): 17-26. doi: 10.12376/j.issn.2097-0501.2023.01.003
HUANG Shenshen, QIU Jiayong, MAO Yimin. Chronic Thromboembolic Pulmonary Hypertension and ChronicThromboembolic Pulmonary Disease[J]. Journal of Rare Diseases, 2023, 2(1): 17-26. doi: 10.12376/j.issn.2097-0501.2023.01.003
Citation: HUANG Shenshen, QIU Jiayong, MAO Yimin. Chronic Thromboembolic Pulmonary Hypertension and ChronicThromboembolic Pulmonary Disease[J]. Journal of Rare Diseases, 2023, 2(1): 17-26. doi: 10.12376/j.issn.2097-0501.2023.01.003

慢性血栓栓塞性肺动脉高压和慢性血栓栓塞性肺疾病

doi: 10.12376/j.issn.2097-0501.2023.01.003
基金项目: 

河南省自然科学基金 182300410365

详细信息
    通信作者:

    毛毅敏,E-mail: 1419295143qq.com

  • 中图分类号: R544.1;R563.5

Chronic Thromboembolic Pulmonary Hypertension and ChronicThromboembolic Pulmonary Disease

Funding: 

The Natural Science Foundation of Henan Province 182300410365

More Information
  • 摘要: 慢性血栓栓塞性肺动脉高压(CTEPH)和慢性血栓栓塞性肺疾病(CTEPD)是急性肺栓塞后因肺动脉内血栓残留、机化,伴或不伴肺动脉高压(PH)的一类疾病。随着诊疗的不断进步、综合治疗策略的观念更新,使这类疾病的预后得到极大改善。但医务人员对这类疾病认识不足,且中国缺少流行病学数据,发病机制未明,具有非特异性临床表现。因此,在临床上易误诊、漏诊和延迟诊断。为了加强医务工作者对其认识,本文对CTEPH和CTEPD的定义、流行病学、病理、发病机制、诊断和治疗等方面予以归纳总结。

     

  • 图  1  疑诊肺动脉高压的诊断策略

    a警告信号包括症状进展迅速、运动耐量严重减弱、轻度运动时出现晕厥前症状或晕厥、右心衰竭的迹象;bCTEPH危险因素:PTE病史、永久性血管内装置、炎症性肠病、原发性血小板增多症、脾切除术、大剂量甲状腺激素替代疗法及恶性肿瘤;PH:肺动脉高压;PAH:动脉型肺动脉高压;NT-proBNP:N端脑钠肽前体;CTEPH:慢性血栓栓塞性肺动脉高压;CTPA:CT肺动脉造影

    Figure  1.  Diagnostic strategy of suspected pulmonary hypertension (PH)

    图  2  肺血栓栓塞症(PTE)后慢性血栓栓塞性肺疾病(CTEPD)和慢性血栓栓塞性肺动脉高压(CTEPH)的诊断策略

    V/Q:核素肺灌注显像;RHC:静息状态下右心导管检查

    Figure  2.  Diagnostic strategies of chronic thrombo-embolic pulmonary disease(CTEPD) and chronic thromboembolic pulmonary hypertension(CTEPH) after pulmonary thromboembolism(PTE)

    图  3  Leiden CTEPH排除流程

    右心室压力过载的心电图标准: 1.V1导联rSR′或rSr′; 2.V1导联R∶S>1,伴有R>0.5 mV; 3.QRS轴>90 °

    Figure  3.  Leiden CTEPH exclusion process

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  • 收稿日期:  2022-12-08
  • 修回日期:  2022-12-31
  • 网络出版日期:  2023-03-07

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