Pathophysiology and new advances in pulmonary hypertension.
Pathophysiology and new advances in pulmonary hypertension.
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DOI：
10.1136/bmjmed-2022-000137
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发表时间：
2023
期刊：
影响因子：
--
通讯作者：
Lahm, Tim
中科院分区：
文献类型：
--
作者：
Bousseau, Simon;Fais, Rafael Sobrano;Gu, Sue;Frump, Andrea;Lahm, Tim
肺动脉高压是一种进行性且常常致命的心肺疾病，其特征是肺动脉压升高、肺循环结构改变以及血管闭塞病变的形成。这些变化导致右心室后负荷增加，通常会进展为适应不良的右心室重塑并最终导致死亡。肺动脉高压是最严重、研究最深入的肺动脉高压类型之一，一直是药物治疗的目标。肺动脉高压的潜在分子发病机制是一个复杂的多因素过程，但可以具有几个特征：炎症、血管生成受损、代谢改变、遗传或表观遗传异常、性和性激素的影响以及右心室异常。目前对肺动脉高压和其他一些类型的肺动脉高压的治疗主要针对参与控制肺血管张力和增殖的途径；然而，这些治疗对患者的治疗效果有限。这篇综述描述了肺动脉高压的主要特征，讨论了当前和新兴的治疗干预措施，并指出了未来的研究和患者护理方向。由于该专业的大部分进展是在肺动脉高压方面取得的，因此本综述重点关注这种类型的肺动脉高压。该综述强调了关键的病理生理学概念和新兴的治疗方向，针对炎症、细胞代谢、遗传学和表观遗传学、性激素信号传导、骨形态发生蛋白信号传导和酪氨酸激酶受体的抑制。
Pulmonary hypertension is a progressive and often fatal cardiopulmonary condition characterised by increased pulmonary arterial pressure, structural changes in the pulmonary circulation, and the formation of vaso-occlusive lesions. These changes lead to increased right ventricular afterload, which often progresses to maladaptive right ventricular remodelling and eventually death. Pulmonary arterial hypertension represents one of the most severe and best studied types of pulmonary hypertension and is consistently targeted by drug treatments. The underlying molecular pathogenesis of pulmonary hypertension is a complex and multifactorial process, but can be characterised by several hallmarks: inflammation, impaired angiogenesis, metabolic alterations, genetic or epigenetic abnormalities, influence of sex and sex hormones, and abnormalities in the right ventricle. Current treatments for pulmonary arterial hypertension and some other types of pulmonary hypertension target pathways involved in the control of pulmonary vascular tone and proliferation; however, these treatments have limited efficacy on patient outcomes. This review describes key features of pulmonary hypertension, discusses current and emerging therapeutic interventions, and points to future directions for research and patient care. Because most progress in the specialty has been made in pulmonary arterial hypertension, this review focuses on this type of pulmonary hypertension. The review highlights key pathophysiological concepts and emerging therapeutic directions, targeting inflammation, cellular metabolism, genetics and epigenetics, sex hormone signalling, bone morphogenetic protein signalling, and inhibition of tyrosine kinase receptors.
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较低的DHEA-S水平可以预测特发性，结缔组织疾病和先天性心脏病相关的肺动脉高压的绝经后妇女的疾病和恶化。
DOI：
10.1183/13993003.00467-2018
发表时间：
2018-06
期刊：
The European respiratory journal
影响因子：
--
作者：
Baird GL;Archer-Chicko C;Barr RG;Bluemke DA;Foderaro AE;Fritz JS;Hill NS;Kawut SM;Klinger JR;Lima JAC;Mullin CJ;Ouyang P;Palevsky HI;Palmisicano AJ;Pinder D;Preston IR;Roberts KE;Smith KA;Walsh T;Whittenhall M;Ventetuolo CE
通讯作者：
Ventetuolo CE
影响因子：
24.3
作者：
Campo, A.;Mathai, S. C.;Hassoun, P. M.
通讯作者：
Hassoun, P. M.
影响因子：
4.4
作者：
Belenkie, I;Smith, ER;Tyberg, JV
通讯作者：
Tyberg, JV
DOI：
10.1183/09031936.00010409
发表时间：
2009-11
期刊：
The European respiratory journal
影响因子：
--
作者：
Austin ED;Cogan JD;West JD;Hedges LK;Hamid R;Dawson EP;Wheeler LA;Parl FF;Loyd JE;Phillips JA 3rd
通讯作者：
Phillips JA 3rd
影响因子：
5.4
作者：
Brittain EL;Niswender K;Agrawal V;Chen X;Fan R;Pugh ME;Rice TW;Robbins IM;Song H;Thompson C;Ye F;Yu C;Zhu H;West J;Newman JH;Hemnes AR
通讯作者：
Hemnes AR