Donaldson Sahai, Ogunti Richard, Kibreab Angesom, Mehari Alem
Howard University College of Medicine, Washington, DC, USA.
Division of Pulmonary and Critical Care, Washington, DC, USA.
Core Evid. 2020 Aug 25;15:31-40. doi: 10.2147/CE.S172791. eCollection 2020.
Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group-4 pulmonary hypertension caused by organized thrombi in pulmonary arteries and vasculopathy in nonoccluded areas leading to right heart failure and death. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy (PEA), which is the guideline recommended treatment. There is increasing experience with balloon pulmonary angioplasty (BPA) for inoperable patients; this option, like PEA, is reserved for specialized centers with expertise in this treatment method. Inoperable patients are candidates for targeted drug therapy. Riociguat remains the only approved medical therapy for CTEPH patients deemed inoperable or with persistent pulmonary hypertension after PEA. The role of riociguat therapy preoperatively or in tandem with BPA is currently under investigation. The purpose of this review is to evaluate the safety and efficacy of riociguat in the treatment of CTEPH.
慢性血栓栓塞性肺动脉高压（CTEPH）被归类为4组肺动脉高压，由肺动脉内的机化血栓和非阻塞区域的血管病变引起，导致右心衰竭和死亡。除了长期抗凝治疗外，每位CTEPH患者都应接受治疗评估，首先评估肺动脉内膜剥脱术（PEA），这是指南推荐的治疗方法。对于无法手术的患者，球囊肺动脉血管成形术（BPA）的经验越来越多；与PEA一样，这种选择仅适用于在该治疗方法方面具有专业知识的专业中心。无法手术的患者是靶向药物治疗的候选者。利奥西呱仍然是唯一被批准用于被认为无法手术或PEA后仍有持续性肺动脉高压的CTEPH患者的药物治疗。目前正在研究利奥西呱术前治疗或与BPA联合治疗的作用。本综述的目的是评估利奥西呱治疗CTEPH的安全性和有效性。
