Yoh A Day & Zimmermann Company, Philadelphia, PA, USA.
Clinical Pharmacy Specialist, Cardiology, University of North Carolina Medical, Chapel Hill, NC, USA.
J Pharm Pract. 2022 Jun;35(3):437-444. doi: 10.1177/0897190020961291. Epub 2020 Oct 21.
Pulmonary hypertension (PH), which includes pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), is a progressive condition with significant morbidity and mortality due to right heart failure if left untreated. Riociguat is a soluble guanylate cyclase (sGC) stimulator and is the only treatment approved for both PAH and CTEPH. The objectives of this review are to describe the epidemiology and pathophysiology of PAH and CTEPH; synthesize the pharmacology, efficacy, safety, and utilization of riociguat; and discuss the role of the pharmacist in managing patients with these conditions. Data presented in this review is supported by peer reviewed literature, using PubMed and key words including pulmonary hypertension, pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and riociguat. The review draws on key studies and review articles that discuss the pathophysiology of PAH and CTEPH, as well as articles discussing the safety and efficacy of riociguat. The overall goal in the treatment of PAH and CTEPH is to improve long-term survival. Treatment planning depends on the type of PH, treatment goals, comorbidities, and risk profiles. Pharmacists serve a valuable role as part of the multidisciplinary team in the care of patients with PH, many of whom may have comorbidities that contribute to high costs and resource utilization. Riociguat is a first-in-class medication and the only approved treatment for both PAH and CTEPH. In clinical trials, riociguat has demonstrated favorable efficacy and tolerability. Riociguat is a valuable addition to the armamentarium of options for treating patients with PH.
肺动脉高压(PH),包括肺动脉高压(PAH)和慢性血栓栓塞性肺动脉高压(CTEPH),如果不治疗,会因右心衰竭而导致发病率和死亡率显著增加。利奥西呱是一种可溶性鸟苷酸环化酶(sGC)刺激剂,是唯一批准用于 PAH 和 CTEPH 的治疗药物。本综述的目的是描述 PAH 和 CTEPH 的流行病学和病理生理学;综合利奥西呱的药理学、疗效、安全性和应用;并讨论药师在管理这些疾病患者中的作用。本综述中提出的数据来自同行评议的文献,使用 PubMed 并使用了包括肺动脉高压、肺动脉高压、慢性血栓栓塞性肺动脉高压和利奥西呱等关键词。本综述借鉴了讨论 PAH 和 CTEPH 病理生理学的关键研究和综述文章,以及讨论利奥西呱安全性和疗效的文章。治疗 PAH 和 CTEPH 的总体目标是提高长期生存率。治疗计划取决于 PH 的类型、治疗目标、合并症和风险状况。药师在 PH 患者的多学科团队护理中发挥着重要作用,许多患者可能存在合并症,导致费用高和资源利用高。利奥西呱是一种首创药物,也是唯一批准用于治疗 PAH 和 CTEPH 的药物。在临床试验中,利奥西呱显示出良好的疗效和耐受性。利奥西呱是治疗 PH 患者的治疗选择的宝贵补充。