Abstract：
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension characterized by the presence of organized thrombi that obstruct pulmonary arteries, ultimately leading to right heart failure and death. Among others, impaired angiogenesis and inflammatory thrombosis have been shown to contribute to the progression of CTEPH. In this review, we summarize the 2-faced nature of angiogenesis in both thrombus formation and resolution in the context of CTEPH and highlight the dual role of angiogenesis and neovascularization in resolving venous thrombi. Furthermore, we discuss relevant in vitro and in vivo models that support the benefits or drawbacks of angiogenesis in CTEPH progression. We discuss the key pathways involved in modulating angiogenesis, particularly the underexplored role of TGFβ (transforming growth factor-beta) signaling in driving fibrosis as an integral element of CTEPH pathogenesis. We finally explore innovative treatment strategies that target angiogenic pathways. These strategies have the potential to pioneer preventive, inventive, or alternative therapeutic options for patients with CTEPH who may not qualify for surgical interventions. Moreover, they could be used synergistically with established treatments such as pulmonary endarterectomy or balloon pulmonary angioplasty. In summary, this review emphasizes the crucial role of angiogenesis in the development of in fibrothrombotic tissue, a major pathological characteristic of CTEPH.
摘要：
慢性血栓栓塞性肺动脉高压（CTEPH）是一种罕见的肺动脉高压，其特征是存在阻塞肺动脉的有组织血栓，最终导致右心衰竭和死亡。其中,受损的血管生成和炎症血栓形成已被证明有助于CTEPH的进展。在这次审查中,我们总结了CTEPH背景下血管生成在血栓形成和消退中的两面性,并强调了血管生成和新生血管形成在解决静脉血栓中的双重作用.此外，我们讨论了相关的体外和体内模型，这些模型支持血管生成在CTEPH进展中的利弊。我们讨论了调节血管生成的关键途径，特别是TGFβ（转化生长因子-β）信号在驱动纤维化中的作用不足作为CTEPH发病机制的组成部分。我们最终探索了靶向血管生成途径的创新治疗策略。这些策略有可能成为预防性的先驱，创造性的,或可能不符合手术条件的CTEPH患者的替代治疗选择。此外，它们可以与已建立的治疗方法协同使用，例如肺内膜切除术或球囊肺血管成形术。总之,这篇综述强调了血管生成在纤维血栓形成组织发展中的关键作用，CTEPH的主要病理特征。
