慢性血栓性肺动脉高压（CTEPH）是一种罕见的进行性肺血管疾病肺动脉高压（PH），在目前PH分类中被归为四型。其诊断依靠右心导管检查测定的肺动脉平均压≥25 mmHg（1 mmHg=0.133 kPa），肺动脉楔压≤15 mmHg，伴有与通气显像不匹配的呈肺段分布的灌注缺损，并已经规律抗凝治疗至少 3个月。CTEPH发病率较低，为每年每百万人口4例。虽然近年对CTEPH的研究深入、临床诊断意识的加强和肺动脉内膜剥脱术，球囊肺血管成形术等治疗方法的出现，CTEPH 患者的预后得到了极大改善。但是CTEPH发病机制不清楚，缺乏有效的早期诊断方法成为限制临床诊疗水平提高的瓶颈。本文将对目前CTEPH发病机制研究进展进行综述，以增加对CTPTH疾病的认识。
Chronic thrombotic pulmonary hypertension (CTEPH) is a rare progressive pulmonary vascular disease, which is classified as World Health Organization (WHO) functional class Ⅳ pulmonary arterial hypertension (PAH). The diagnosis was based on mean pulmonary arterial pressure ≥25 mmHg(1 mmHg=0.133 kPa) and pulmonary arterial wedge pressure ≤15 mmHg measured by right heart catheterization, with the under-utilization of radionuclide ventilation/perfusion scan and regular anticoagulation therapy for at least three months. The incidence of CTEPH is low, at 4 cases per million population per year. Despite the in-depth research on CTEPH in recent years, the strengthening of clinical diagnostic awareness, and the emergence of treatment methods such as pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA), the prognosis of CTEPH patients has been dramatically improved. However, the pathogenesis of CTEPH is unclear, and the lack of effective early diagnostic methods has become a bottleneck limiting the improvement of clinical diagnosis and treatment. In this paper, we reviewed the current research progress on the pathogenesis of CTEPH to increase the understanding of CTPTH disease.
陈奕霖,张婷婷,陈豫钦,等. 慢性血栓栓塞性肺动脉高压发病机制研究进展[J]. 中华结核和呼吸杂志,2022,45(03)：304-308.
DOI:10.3760/cma.j.cn112147-20220106-00019版权归中华医学会所有。
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陈奕霖, 张婷婷, 陈豫钦, 等. 慢性血栓栓塞性肺动脉高压发病机制研究进展[J]. 中华结核和呼吸杂志, 2022, 45(3): 304-308. DOI: 10.3760/cma.j.cn112147-20220106-00019.
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