Division of Pulmonary and Critical Care, Massachusetts General Hospital, Boston, MA, USA.
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA.
Curr Cardiol Rep. 2021 Aug 19;23(10):141. doi: 10.1007/s11886-021-01572-6.
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.
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. 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.
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，在这种情况下，富含血小板的红色血栓不会溶解，而是在肺血管中形成一种有组织的黄色、纤维化的瘢痕样阻塞。在这里，我们回顾了 CTEPH 的病理生物学。
我们目前的知识主要来自于对人类样本和动物模型的研究，但这些研究在重现疾病所有方面的能力上存在固有局限性。这些研究已经确定了血小板生物学和炎症在形成包含内皮细胞、肌成纤维细胞和免疫细胞的瘢痕样血栓中的改变，以及小血管肺动脉高压样血管病变。目前，对 CTEPH 特定治疗方法的了解有限，阻碍了其发展。开发新的 CTEPH 医学治疗方法需要对其病理生物学有新的认识，从而弥合从实验室到临床的差距。
