University Cardiology Division, Pisa University Hospital and University of Pisa, Via Paradisa, 2, Pisa 56124, Italy.
University Cardiology Division, Pisa University Hospital and University of Pisa, Via Paradisa, 2, Pisa 56124, Italy.
Eur J Intern Med. 2024 Nov;129:1-15. doi: 10.1016/j.ejim.2024.07.030. Epub 2024 Aug 1.
Pulmonary hypertension associated to left heart disease (PH-LHD) refers to a clinical and haemodynamic condition of pulmonary hypertension associated with a heterogeneous group of diseases affecting any of the compartments that form the left ventricle and left atrium. PH-LHD is the most common cause of PH, accounting for 65-80 % of diagnoses. Based on the haemodynamic phase of the disease, PH-LDH is classified into three subgroups: postcapillary PH, isolated postcapillary PH and combined pre-postcapillary PH (CpcPH). Several signaling pathways involved in the regulation of vascular tone are dysfunctional in PH-LHD, including nitric oxide, MAP kinase and endothelin-1 pathways. These pathways are the same as those altered in PH group 1, however PH-LHD can heardly be treated by specific drugs that act on the pulmonary circulation. In this manuscript we provide a state of the art of the available clinical trials investigating the safety and efficacy of PAH-specific drugs, as well as drugs active in patients with heart failure and PH-LHD. We also discuss the different phenotypes of PH-LHD, as well as molecular targets and signaling pathways potentially involved in the pathophysiology of the disease. Finally we will mention some new emerging therapies that can be used to treat this form of PH.
与左心疾病相关的肺动脉高压（PH-LHD）是指一种与影响左心室和左心房任何部位的异质性疾病相关的肺动脉高压的临床和血液动力学状态。PH-LHD 是 PH 最常见的原因，占诊断的 65-80%。根据疾病的血液动力学阶段，PH-LDH 分为三组：毛细血管后 PH、孤立性毛细血管后 PH 和混合前毛细血管后 PH（CpcPH）。在 PH-LHD 中，参与血管张力调节的几个信号通路功能失调，包括一氧化氮、MAP 激酶和内皮素-1 通路。这些通路与 PH 组 1 中改变的通路相同，但 PH-LHD 几乎不能用作用于肺循环的特定药物治疗。在本文中，我们提供了关于正在进行的临床试验的最新情况，这些试验调查了 PAH 特异性药物以及对心力衰竭和 PH-LHD 患者有效的药物的安全性和疗效。我们还讨论了 PH-LHD 的不同表型，以及可能参与疾病病理生理学的分子靶点和信号通路。最后，我们将提到一些可用于治疗这种 PH 形式的新出现的治疗方法。
