Background. There is significant controversy relating to whether chronic thromboembolic pulmonary hypertension (CTEPH) can be treated with pulmonary arterial hypertension- (PAH-) targeted therapies and which therapy is the optimal choice for patients. A large number of randomized controlled trials (RCTs) have compared PAH-targeted therapies with placebo or conventional therapies. In this study, we aimed to compare all of the PAH-targeted medications that are used to treat CTEPH and rank their efficacy by the application of network meta-analysis (NMA). Methods. We searched PubMed, EMBASE, Web of Science, the Cochrane Central Register, https://clinicaltrials.gov, and who.int/trialsearch/, for relevant RCTs published up to January 2020. In addition to traditional meta-analysis, we also performed NMA in our systematic review, as deployed in a previous protocol (PROSPERO: CRD42020173765). Results. Our study identified eight eligible RCTs that evaluated seven PAH-targeted therapies in 703 patients with CTEPH. NMA revealed that riociguat was ranked first as the most optimized therapy for ameliorating the 6-minute walk distance with a probability of 80.4%. Bosentan was significantly better than others with regard to reducing brain natriuretic peptide/N-terminal pro-B-type natriuretic peptide with a probability of 84.3%. Sildenafil was identified as the best drug in terms of improving the New York Heart Association/World Health Organization functional class with a probability of 87.3%. Treprostinil and macitentan were more beneficial than other drugs in reducing pulmonary vascular resistance and lowering the incidence of clinical worsening with probabilities of 86.2% and 79.2%, respectively. Conclusion. Analysis revealed positive advantages for the use of PAH-targeted drugs in patients with CTEPH. Overall, treprostinil and riociguat were superior to all other PAH-targeted medications in most of the outcomes investigated.

中文翻译：

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慢性血栓栓塞性肺动脉高压靶向治疗的疗效和安全性比较：系统评价和网络荟萃分析

背景。关于慢性血栓栓塞性肺动脉高压（CTEPH）是否可以用肺动脉高压（PAH-）靶向治疗以及哪种治疗是患者的最佳选择存在重大争议。大量随机对照试验 (RCT) 将 PAH 靶向疗法与安慰剂或常规疗法进行了比较。在本研究中，我们旨在比较所有用于治疗 CTEPH 的 PAH 靶向药物，并通过应用网络荟萃分析 (NMA) 对其疗效进行排名。方法. 我们在 PubMed、EMBASE、Web of Science、Cochrane Central Register、https://clinicaltrials.gov 和 who.int/trialsearch/ 中搜索了截至 2020 年 1 月发布的相关 RCT。除了传统的荟萃分析，我们还在我们的系统评价中执行了 NMA，如之前的协议（PROSPERO：CRD42020173765）中所部署的。结果. 我们的研究确定了 8 项符合条件的 RCT，评估了 703 名 CTEPH 患者的 7 项 PAH 靶向疗法。NMA 显示，riociguat 以 80.4% 的概率被列为改善 6 分钟步行距离的最优化疗法。波生坦在降低脑钠肽/N端前B型钠尿肽方面明显优于其他药物，概率为84.3%。西地那非被确定为改善纽约心脏协会/世界卫生组织功能等级的最佳药物，概率为 87.3%。曲前列环素和马西替坦在降低肺血管阻力和降低临床恶化发生率方面比其他药物更有益，概率分别为86.2%和79.2%。结论. 分析揭示了在 CTEPH 患者中使用 PAH 靶向药物的积极优势。总体而言，在大多数调查的结果中，曲前列环素和利奥西呱优于所有其他 PAH 靶向药物。