Sun Lu, Tian Han, Liu Jixiang, Liu Min, Li Yuhan, Li Haobo, Miao Ran, Zhang Yunxia, Xie Wanmu, Zhang Zhu, Xu Shiqing, Yang Peiran, Zhai Zhenguo
China-Japan Friendship Hospital Chinese Academy of Medical Sciences & Peking Union Medical College Beijing China.
National Center for Respiratory Medicine; State Key Laboratory of Respiratory Health and Multimorbidity; National Clinical Research Center for Respiratory Diseases; Institute of Respiratory Medicine, Chinese Academy of Medical Sciences; Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine China-Japan Friendship Hospital Beijing China.
Pulm Circ. 2025 Aug 1;15(3):e70118. doi: 10.1002/pul2.70118. eCollection 2025 Jul.
Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by persistent obstruction and vascular remodeling of the pulmonary arteries following pulmonary thromboembolism (PTE), diagnosed after a minimum of 3 months of therapeutic anticoagulation. Disease progression from PTE to CTEPH takes place in the pulmonary circulation, where the vascular microenvironment is composed of a fluid portion that includes blood cells and components of the fibrinolytic system, and various vascular cells, including endothelial and smooth muscle cells. Following PTE, the homeostasis of the pulmonary vascular microenvironment is disrupted, leading to the accumulation of inflammatory mediators and immune cells at the site of thrombosis. Platelets are also involved in the regulation of coagulation and inflammation, and functional changes such as impaired fibrinolysis are observed. Subsequently, endothelial cell dysfunction and smooth muscle cell dysregulation lead to delayed thrombus resolution and pulmonary vascular remodeling, eventually resulting in CTEPH. Early intervention targeting the aberrant vascular microenvironment may thwart or mitigate the transition from PTE to CTEPH. Here, we discuss the development of CTEPH from the perspective of the pulmonary vascular microenvironment and examine its related biomarkers and therapeutic methods for CTEPH diagnosis and treatment.
慢性血栓栓塞性肺动脉高压（CTEPH）的特征是肺血栓栓塞（PTE）后肺动脉持续阻塞和血管重塑，在至少3个月的治疗性抗凝后诊断。从PTE到CTEPH的疾病进展发生在肺循环中，其中血管微环境由包括血细胞和纤维蛋白溶解系统成分的液体部分以及包括内皮细胞和平滑肌细胞在内的各种血管细胞组成。PTE后，肺血管微环境的稳态被破坏，导致血栓形成部位炎症介质和免疫细胞的积累。血小板也参与凝血和炎症的调节，并观察到诸如纤维蛋白溶解受损等功能变化。随后，内皮细胞功能障碍和平滑肌细胞失调导致血栓溶解延迟和肺血管重塑，最终导致CTEPH。针对异常血管微环境的早期干预可能会阻止或减轻从PTE到CTEPH的转变。在此，我们从肺血管微环境的角度讨论CTEPH的发展，并研究其相关生物标志物以及用于CTEPH诊断和治疗的方法。
