Estrada Rodolfo A, Sahay Sandeep, Tonelli Adriano R
Division of Pulmonary Diseases and Critical Care Medicine, UT Health San Antonio, San Antonio, TX, USA.
Division of Pulmonary, Critical Care & Sleep Medicine, Houston Methodist Hospital, Houston, TX, USA.
Ther Adv Respir Dis. 2025 Jan-Dec;19:17534666251342898. doi: 10.1177/17534666251342898. Epub 2025 May 23.
This review focuses on the advancements in the treatment of pulmonary hypertension (PH), especially after the Food and Drug Administration (FDA) approval of sotatercept and the advances in treatment recommendations after seven World Symposia on PH. PH, a complex and progressive condition defined hemodynamically by a mean pulmonary artery pressure >20 mmHg, encompasses multiple PH groups, each with distinct pathophysiological characteristics and treatment implications. Diagnosing PH can be challenging because symptoms like shortness of breath, fatigue, and chest pain are nonspecific. Contemporary treatment of pulmonary arterial hypertension aims to improve outcomes, symptoms, and overall quality of life, with a primary focus on preventing and treating right ventricular failure. Comprehensive risk stratification remains crucial, aiding in personalized therapy adjustments that improve patients' outcomes. This review also touches upon the limited treatment options for other PH groups, like PH associated with left heart disease, parenchymal lung diseases, and chronic thromboembolic PH, underscoring the need for expanded therapeutic options. Despite advances, challenges remain: diagnostic delays, misdiagnosis, absence of head-to-head clinical trials, and the timing of introducing newer treatments such as sotatercept are discussed, emphasizing an integrated approach that transcends vasodilation to target underlying disease mechanisms. Future directions envision a comprehensive risk stratification incorporating right ventricular function and a mechanism-based treatment paradigm, encouraging a tailored therapeutic approach in PH management.
本综述聚焦于肺动脉高压(PH)治疗方面的进展,尤其是在美国食品药品监督管理局(FDA)批准索他瑞西普之后以及七届世界肺动脉高压研讨会之后治疗建议的进展。PH是一种复杂的进行性疾病,通过平均肺动脉压>20 mmHg进行血流动力学定义,涵盖多个PH组,每组都有独特的病理生理特征和治疗意义。诊断PH可能具有挑战性,因为诸如呼吸急促、疲劳和胸痛等症状并不具有特异性。当代肺动脉高压治疗旨在改善预后、症状和整体生活质量,主要侧重于预防和治疗右心室衰竭。全面的风险分层仍然至关重要,有助于进行个性化治疗调整以改善患者预后。本综述还涉及其他PH组的有限治疗选择,如与左心疾病、实质性肺疾病和慢性血栓栓塞性PH相关的PH,强调需要扩大治疗选择。尽管取得了进展,但挑战依然存在:讨论了诊断延迟、误诊、缺乏头对头临床试验以及引入索他瑞西普等新治疗方法的时机,强调了一种超越血管舒张以针对潜在疾病机制的综合方法。未来方向设想采用一种综合风险分层方法,纳入右心室功能和基于机制的治疗模式,鼓励在PH管理中采用量身定制的治疗方法。