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

Chronic Thromboembolic Pulmonary Hypertension and ChronicThromboembolic Pulmonary Disease

  • 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。

     

    Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic pulmonary disease (CTEPD) are diseases that occur after acute pulmonary embolism resulting from residual and organized intra-pulmonary thrombus, with or without pulmonary hypertension(PH). The prognosis of these diseases has been dramatically improved with the continuous progress of treatment and the updated concept of the comprehensive treatment strategy. However, there is a lack of awareness of these diseases among medical practitioners, and a lack of epidemiological data in China, with unknown pathogenesis and non-specific clinical manifestations. Therefore, it is easy to misdiagnosis, underdiagnosis and delay diagnosis in clinical practice. To enhance the understanding of medical personnel, this paper summarizes the definition, epidemiology, pathology, pathogenesis, diagnosis and treatment of CTEPH and CTEPD.

     

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