Pulmonary arterial hypertension: the clinical syndrome.
Pulmonary arterial hypertension: the clinical syndrome.
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DOI：
10.1161/circresaha.115.301146
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发表时间：
2014-06-20
影响因子：
20.1
通讯作者：
Gladwin MT
中科院分区：
文献类型：
--
作者：
Lai YC;Potoka KC;Champion HC;Mora AL;Gladwin MT
肺动脉高压（PAH）是一种进行性疾病，内皮功能障碍和血管重构阻塞小肺动脉，导致肺血管阻力和肺压力增加。这将导致心输出量减少，右心衰，最终死亡。在这篇综述中，我们试图回答一些被诊断为PAH的患者常问的与该疾病有关的重要问题，并旨在从分类、诊断、病理生理学、遗传病因、人口统计学和预后因素方面提供解释。此外，本文还综述了在PAH发病机制中起核心作用的重要分子途径，包括一氧化氮、前列腺环素、内皮素-1、活性氧以及内皮和平滑肌增殖。
Pulmonary arterial hypertension (PAH) is a progressive disorder in which endothelial dysfunction and vascular remodeling obstruct small pulmonary arteries, resulting in increased pulmonary vascular resistance and pulmonary pressures. This leads to reduced cardiac output, right heart failure, and ultimately death. In this review, we attempt to answer some important questions commonly asked by patients diagnosed with PAH pertaining to the disease, and aim to provide an explanation in terms of classification, diagnosis, pathophysiology, genetic etiologies, demographics, and prognostic factors. Furthermore, important molecular pathways that are central to the pathogenesis of PAH are reviewed, including nitric oxide, prostacyclin, endothelin-1, reactive oxygen species, and endothelial and smooth muscle proliferation.