Purpose of Review

Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon complication of acute pulmonary embolism (PE), in which the red, platelet-rich thrombus does not resolve but forms into an organized yellow, fibrotic scar-like obstruction in the pulmonary vasculature. Here we review the pathobiology of CTEPH.

Recent Findings

Our current knowledge has predominantly been informed by studies of human samples and animal models that are inherently limited in their ability to recapitulate all aspects of the disease. These studies have identified alterations in platelet biology and inflammation in the formation of a scar-like thrombus that comprised endothelial cells, myofibroblasts, and immune cells, along with a small vessel pulmonary arterial hypertension-like vasculopathy.

Summary

The development of CTEPH-specific therapies is currently hindered by a limited knowledge of its pathobiology. The development of new CTEPH medical therapies will require new insights into its pathobiology that bridge the gap from bench to bedside.

中文翻译：

---

慢性血栓栓塞性肺动脉高压：替补

审查目的

慢性血栓栓塞性肺动脉高压 (CTEPH) 是急性肺栓塞 (PE) 的一种罕见并发症，其中富含血小板的红色血栓不会消退，而是在肺血管系统中形成有组织的黄色纤维化瘢痕样阻塞。在这里，我们回顾了 CTEPH 的病理生物学。

最近的发现

我们目前的知识主要来自对人类样本和动物模型的研究，这些研究在概括疾病各个方面的能力方面存在固有的局限性。这些研究已经确定了血小板生物学的改变和炎症在瘢痕样血栓形成中的变化，瘢痕样血栓包括内皮细胞、肌成纤维细胞和免疫细胞，以及小血管性肺动脉高压样血管病变。

概括

CTEPH 特异性疗法的发展目前受到其病理生物学知识有限的阻碍。新 CTEPH 药物疗法的开发将需要对其病理生物学的新见解，以弥合从实验室到床边的差距。