Abstract：
Chronic thromboembolic pulmonary hypertension (CTEPH) is thought to occur as a sequelae of thromboembolic processes in the pulmonary vasculature. The pathophysiology of CTEPH is multifactorial, including impaired fibrinolysis, endothelial dysregulation, and hypoxic adaptations. The diagnosis of CTEPH is typically delayed considering the nonspecific nature of the symptoms, lack of screening, and relatively low incidence. Diagnostic tools include ventilation-perfusion testing, echocardiography, cardiac catheterization, and pulmonary angiography. The only potentially curative treatment for CTEPH is pulmonary endarterectomy However, approximately 40% of patients are inoperable. Currently, only Riociguat is Food and Drug Administration approved specifically for CTEPH, with additional drug trials underway.
摘要：
慢性血栓栓塞性肺动脉高压（CTEPH）被认为是肺血管系统中血栓栓塞过程的后遗症。CTEPH的病理生理学是多因素的，包括受损的纤维蛋白溶解,内皮失调，和低氧适应。考虑到症状的非特异性，CTEPH的诊断通常会延迟。缺乏筛查,发病率相对较低。诊断工具包括通气灌注测试，超声心动图,心导管插入术,还有肺动脉造影.CTEPH的唯一潜在治疗方法是肺内膜切除术。大约40%的患者无法手术。目前,只有Riociguat被食品和药物管理局专门批准用于CTEPH，正在进行其他药物试验。
