Department of Cardiovascular and Respiratory Science, Sapienza University of Rome, viale del Policlinico 155, 00161, Rome, Italy.
Curr Heart Fail Rep. 2023 Aug;20(4):263-270. doi: 10.1007/s11897-023-00612-2. Epub 2023 Jul 24.
The purpose of this review is to overview the most relevant and recent knowledge regarding medical management in pulmonary arterial hypertension (PAH).
Evidence has shown that PAH patients' quality of life and prognosis depend on the capability of the RV to adapt to increased afterload and to fully recover in response to substantially reduced pulmonary vascular resistance obtained with medical therapy. Data from recent clinical studies show that more aggressive treatment strategies, especially in higher risk categories, determine larger afterload reductions, consequentially increasing the probability of achieving right heart reverse remodeling, therefore improving the patients' survival and quality of life. Remarkable progress has been observed over the past decades in the medical treatment of PAH, related to the development of drugs that target multiple biological pathways, strategies for earlier and more aggressive treatment interventions. New hopes for treatment of patients who are unable to achieve low-risk status have been derived from the phase 2 trial PULSAR and the phase 3 trial STELLAR, which show improvement in the hemodynamic status of patients treated with sotatercept on top of background therapy. Promising results are expected from several ongoing clinical trials targeting new pathways involved in the pathophysiology of PAH.
本文旨在综述肺动脉高压（PAH）医学管理方面最相关和最新的知识。
研究表明，PAH 患者的生活质量和预后取决于右心室（RV）适应增加的后负荷的能力，以及通过药物治疗获得的肺血管阻力显著降低后RV 充分恢复的能力。最近的临床研究数据表明，更积极的治疗策略，特别是在更高风险类别中，可实现更大的后负荷降低，从而增加实现右心重构逆转的可能性，进而改善患者的生存和生活质量。在过去几十年中，PAH 的药物治疗取得了显著进展，这与靶向多种生物途径的药物的发展以及更早和更积极的治疗干预策略有关。在背景治疗的基础上，使用 sotatercept 治疗的患者的血流动力学状态得到改善，这一结果来自 PULSAR 二期临床试验和 STELLAR 三期临床试验，为无法达到低危状态的患者的治疗带来了新的希望。针对 PAH 病理生理学中涉及的新途径的几项正在进行的临床试验有望取得有前景的结果。
