Kopeć Grzegorz, Skride Andris, Ereminiene Egle, Simkova Iveta, Enache Roxana, Samarzija Miroslav, Salobir Barbara, Jansa Pavel
Pulmonary Circulation Center, Department of Cardiac and Vascular Diseases, Jagiellonian University Medical College, Kraków, Poland.
St. John Paul II Hospital, Kraków, Poland.
Kardiol Pol. 2025;83(1):18-26. doi: 10.33963/v.phj.104053. Epub 2025 Jan 2.
Pulmonary arterial hypertension (PAH) is a severe and progressive disease with limited survival prospects under currently available therapies. Since the 2022 edition of the European Society of Cardiology and European Respiratory Society guidelines on pulmonary hypertension, substantial clinical evidence has emerged, supporting a new treatment algorithm for PAH as presented at the 7th World Symposium on Pulmonary Hypertension 2024 and the following proceeding papers. Key updates include the introduction of sotatercept as a second-line therapy leading to a revised definition of maximal medical therapy now encompassing agents from four therapeutic groups (phosphodiesterase-5 inhibitors/soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin pathway agents, and sotatercept), instead of three (phosphodiesterase-5 inhibitors/soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin pathway agents). Other novelties include the elimination of a distinct pathway for patients with cardiopulmonary comorbidities in favor of an individualized approach, a reduction in the initial patient assessment risk categories from three to two, and a follow-up interval shortened from 3-6 months to 3-4 months post-treatment initiation. This review presents these advancements and emphasizes the need for their widespread implementation in clinical practice. At the end, we present new opportunities and challenges in the treatment of pulmonary arterial hypertension in eight Central and Eastern European countries.
肺动脉高压(PAH)是一种严重的进行性疾病,在目前可用的治疗方法下生存前景有限。自2022年欧洲心脏病学会和欧洲呼吸学会肺动脉高压指南发布以来,大量临床证据涌现,支持了2024年第七届世界肺动脉高压研讨会及后续论文中提出的PAH新治疗算法。主要更新内容包括引入索他瑞西普作为二线治疗药物,这导致了最大药物治疗的定义修订,现在涵盖来自四个治疗组(磷酸二酯酶-5抑制剂/可溶性鸟苷酸环化酶刺激剂、内皮素受体拮抗剂、前列环素途径药物和索他瑞西普)的药物,而不是三个(磷酸二酯酶-5抑制剂/可溶性鸟苷酸环化酶刺激剂、内皮素受体拮抗剂、前列环素途径药物)。其他新变化包括取消了针对心肺合并症患者的独特治疗途径,转而采用个体化方法,将初始患者评估风险类别从三类减少到两类,并将治疗开始后的随访间隔从3至6个月缩短至3至4个月。本综述介绍了这些进展,并强调了在临床实践中广泛实施这些进展的必要性。最后,我们介绍了中东欧八个国家在肺动脉高压治疗方面的新机遇和挑战。