Pradhan Akshyaya, Tyagi Richa, Sharma Prachi, Bajpai Jyoti, Kant Surya
Department of Cardiology, King George's Medical University Lucknow, Uttar Pradesh, India.
Department of Pulmonary Medicine, Sanjay Gandhi PG Institute of Medical Sciences Lucknow, Uttar Pradesh, India.
Eur Cardiol. 2024 Dec 23;19:e25. doi: 10.15420/ecr.2024.11. eCollection 2024.
Pulmonary arterial hypertension (PAH) is a long-term condition characterised by increased resistance to blood flow in the pulmonary circulation. The disease has a progressive course and is associated with a poor prognosis. Without treatment, PAH is associated with mortality in <3 years. Over the past decade, many advances have been made in revising the haemodynamic definition, clinical classification, risk calculation score, treatment options etc. Suggestions from the Sixth World Symposium on Pulmonary Hypertension were incorporated into a literature review that was included in the European Society of Cardiology/European Respiratory Society (ESC/ERS)'s most recent iteration of their guidelines in 2022. The traditional cut-off for pulmonary hypertension (PH), i.e., mean pulmonary artery pressure (mPAP) >25 mm Hg, has been challenged by observational cohort studies, which have shown poor outcomes for values of 21-24 mmHg; the new consensus is that PH is defined at mPAP >20 mm Hg. Although the gold standard for diagnosis and the major source of therapy guidance continues to be right cardiac catheterisation, echocardiography remains the initial test of choice. A multidisciplinary approach is highly recommended when treating PH patients and careful evaluation of patients will aid in proper diagnosis and prognosis. Pharmacotherapy for PAH has seen a paradigm shift with the successful use of newer agents in more extensive, longer and more inclusive trials driven by hard endpoints. Macitentan, selexipag and riociguat are three oral agents that have shown astounding success in PAH randomised studies in the past decade. Upfront combination therapy with two agents is now becoming the norm (following the AMBITION, OPTIMA and ITALY trials) and the momentum is shifting towards triple therapy as for essential hypertension. More recently, inhaled treprostinil was shown to improve exercise capacity in PH associated with interstitial lung disease in the phase III INCREASE study and has been granted regulatory approval for World Health Organization group 3 PH. A new class of drug, sotatercept (a tumour growth factor-β signalling inhibitor), has also been recently approved by the Food and Drug Administration for management of PAH based on positive results from the phase III STELLAR study. Pulmonary artery denervation and balloon pulmonary angioplasty have emerged as viable alternatives in PH that are resistant to drug therapy. This article aims to summarise the key changes and recent advances in diagnosis and managing PH in general, with an emphasis on certain subgroups.
肺动脉高压（PAH）是一种长期疾病，其特征是肺循环中血流阻力增加。该疾病呈进行性发展，预后较差。未经治疗的PAH患者在3年内的死亡率较高。在过去十年中，在修订血流动力学定义、临床分类、风险计算评分、治疗选择等方面取得了许多进展。第六届世界肺动脉高压研讨会的建议被纳入一篇文献综述，该综述被纳入欧洲心脏病学会/欧洲呼吸学会（ESC/ERS）2022年最新版的指南中。肺动脉高压（PH）的传统阈值，即平均肺动脉压（mPAP）>25 mmHg，已受到观察性队列研究的挑战，这些研究表明，mPAP值在21 - 24 mmHg时预后较差；新的共识是，PH定义为mPAP >20 mmHg。尽管诊断的金标准和治疗指导的主要来源仍然是右心导管检查，但超声心动图仍然是首选的初始检查方法。在治疗PH患者时，强烈推荐采用多学科方法，对患者进行仔细评估将有助于正确诊断和判断预后。随着在更广泛、更长期和更具包容性的试验中成功使用新药物（以硬终点为驱动），PAH的药物治疗发生了范式转变。马昔腾坦、司来帕格和利奥西呱是三种口服药物，在过去十年的PAH随机研究中显示出惊人的成功。现在，两种药物的初始联合治疗正成为常态（继AMBITION、OPTIMA和ITALY试验之后），并且治疗趋势正朝着像原发性高血压那样的三联疗法转变。最近，在III期INCREASE研究中，吸入性曲前列尼尔被证明可改善与间质性肺疾病相关的PH患者的运动能力，并已获得世界卫生组织第3组PH的监管批准。一类新型药物索他瑞西（一种肿瘤生长因子-β信号抑制剂），最近也基于III期STELLAR研究的阳性结果，获得了美国食品药品监督管理局对PAH治疗的批准。肺动脉去神经支配术和球囊肺动脉成形术已成为对药物治疗耐药的PH患者可行的替代治疗方法。本文旨在总结一般PH诊断和管理的关键变化及最新进展，重点关注某些亚组。
