Strategizing Drug Therapies in Pulmonary Hypertension for Improved Outcomes.
Strategizing Drug Therapies in Pulmonary Hypertension for Improved Outcomes.
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DOI：
10.3390/ph15101242
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发表时间：
2022-10-10
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影响因子：
--
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肺动脉高压 (PH) 的特征是静息平均肺动脉压 (PAP) 为 20 mmHg 或更高，并且是一种多种病因的疾病。在各种类型的肺动脉高压中，肺动脉高压 (PAH) 的特点是肺动脉树阻力升高。这是一种罕见但致命的疾病，其特征是远端肺动脉的血管重塑。本文重点讨论 PAH 的诊断和管理，包括当前和未来的治疗方案。在过去的 15 年里，我们对这种进行性疾病的理解已经从肺小动脉血管收缩/血管舒张不匹配的概念扩展到现在对遗传决定因素、众多细胞信号传导途径、细胞增殖和凋亡、纤维化、血栓形成和代谢异常的作用有了更好的认识。虽然对其病理生理学的了解有所扩展，但当今可用的大多数治疗方法仍然调节 30 多年来一直针对的相同的三种血管舒张途径（内皮素、一氧化氮和前列环素）。虽然改变这些途径可能有助于改善症状和生活质量，但这些途径都不能直接改变潜在的疾病发病机制。然而，目前正在进行的新药研究可以预防或逆转 PAH 的这些根本原因。这篇综述讨论了当前 PAH 治疗算法的证据基础，并讨论了正在开发的新疗法。
Pulmonary hypertension (PH) is characterized by a resting mean pulmonary artery pressure (PAP) of 20 mmHg or more and is a disease of multiple etiologies. Of the various types of PH, pulmonary arterial hypertension (PAH) is characterized by elevated resistance in the pulmonary arterial tree. It is a rare but deadly disease characterized by vascular remodeling of the distal pulmonary arteries. This paper focuses on PAH diagnosis and management including current and future treatment options. Over the last 15 years, our understanding of this progressive disease has expanded from the concept of vasoconstrictive/vasodilatory mismatch in the pulmonary arterioles to now a better appreciation of the role of genetic determinants, numerous cell signaling pathways, cell proliferation and apoptosis, fibrosis, thrombosis, and metabolic abnormalities. While knowledge of its pathophysiology has expanded, the majority of the treatments available today still modulate the same three vasodilatory pathways that have been targeted for over 30 years (endothelin, nitric oxide, and prostacyclin). While modifying these pathways may help improve symptoms and quality of life, none of these directly modify the underlying disease pathogenesis. However, there are now studies ongoing with new drugs that can prevent or reverse these underlying causes of PAH. This review discusses the evidence base for the current treatment algorithms for PAH, as well as discusses novel therapies in development.
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影响因子：
5.8
作者：
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影响因子：
158.5
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