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第 3 组肺动脉高压:从基础到临床。

Group 3 Pulmonary Hypertension: From Bench to Bedside.

机构信息

Division of Pulmonary, Critical Care and Sleep Medicine (N.S., C.E.V.), Brown University, Providence, RI.

Department of Pathology, Universities of Giessen and Marburg Lung Center (UGMLC), Justus-Liebig University, Germany (P.D.).

出版信息

Circ Res. 2022 Apr 29;130(9):1404-1422. doi: 10.1161/CIRCRESAHA.121.319970. Epub 2022 Apr 28.

Abstract

Pulmonary hypertension (PH) because of chronic lung disease is categorized as Group 3 PH in the most recent classification system. Prevalence of these diseases is increasing over time, creating a growing need for effective therapeutic options. Recent approval of the first pulmonary arterial hypertension therapy for the treatment of Group 3 PH related to interstitial lung disease represents an encouraging advancement. This review focuses on molecular mechanisms contributing to pulmonary vasculopathy in chronic hypoxia, the pathology and epidemiology of Group 3 PH, the right ventricular dysfunction observed in this population and clinical trial data that inform the use of pulmonary vasodilators in Group 3 PH.

摘要

慢性肺部疾病引起的肺动脉高压(PH)在最新的分类系统中被归类为第 3 组 PH。这些疾病的患病率随着时间的推移而增加,因此需要有效的治疗选择。最近批准了第一种肺动脉高压治疗药物,用于治疗与间质性肺病相关的第 3 组 PH,这是一个令人鼓舞的进展。这篇综述重点介绍了慢性缺氧导致肺血管病变的分子机制、第 3 组 PH 的病理学和流行病学、该人群中观察到的右心室功能障碍以及临床试验数据,这些数据为第 3 组 PH 中使用肺血管扩张剂提供了信息。

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