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慢性血栓栓塞性肺动脉高压(chronic thromboembolic pulmonary hypertension,CTEPH)是一种慢性进展性疾病,如果未经治疗,预后极差.2013年在法国尼斯召开的第五届世界肺动脉高压论坛会上将CTEPH归为第四大类肺动脉高压.由于CTEPH的症状因人而异且缺乏特异性,容易引起漏诊、误诊和延迟诊断.外科肺动脉血栓内膜剥脱术是唯一有可能治愈CTEPH的办法,但有一部分患者属于CTEPH分型中的Ⅲ型和Ⅳ型,即阻塞在亚段以下的远端或者非阻塞部位的小动脉发生血管重塑,使患者手术难度增大甚至不能进行外科手术,还有小部分患者接受外科手术后持续存在肺动脉高压.动脉性肺动脉高压和CTEPH在临床表现、病理特点和发病机制方面有交叉重叠部分,因此推动了有关靶向药物治疗CTEPH患者有效性和安全性的相关研究.“,”Chronic thromboembolic pulmonary hypertension(CTEPH) is a progressive deadly disease with poor prognosis if not treated.It was classified as the Ⅳ type at the 2013 World Symposium on Pulmonary Hypertension in Nice,France.The optimal treatment is surgery with pulmonary endarterectomy.Nevertheless,a significant percentage of patients are deemed non-operable due to distal distribution of the disease and arteriopathy in the non-occluded areas.And another percentage of CTEPH patients remain pulmonary hypertension after pulmonary endarterectomy.The overlap in clinical presentation,pathological features and pathogenesis between pulmonary arterial hypertension(PAH) and CTEPH provides a compelling rationale for exploring the efficacy of PAH-targeted therapies in CTEPH.