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<p>Pulmonary arterial hypertension (PAH) is a progressive and life-limiting condition caused by pulmonary vascular remodeling due to heterogeneous aetiologies. Despite significant advances in treatment, PAH remains incurable and is associated with a poor prognosis, highlighting the need for novel therapeutic options. Recent advances in molecular and translational research have uncovered key pathways involved in PAH, including endothelial dysfunction, smooth muscle cell proliferation, inflammation, and thrombosis. These insights have guided the development of novel therapeutic strategies aimed at modulating these mechanisms. Promising compounds currently under investigation include agents targeting the bone morphogenic protein/transforming growth factor-beta axis, epigenetic modulators, receptor tyrosine kinase inhibitors, and immunomodulatory biologics. In addition, vasodilators acting on the renin–angiotensin–aldosterone system and the soluble guanylate cyclase pathway, as well as metabolic and hormonal modulators are under investigation, with clinical trials showing encouraging results for several agents. This review outlines the current understanding of PAH pathobiology and the most promising emerging treatments for pulmonary hypertension.</p>

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Emerging treatments in pulmonary arterial hypertension: a current review

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年4月29日

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  • Philipp Grubwieser,
  • Nina Bonkat,
  • Birgit Sailer,
  • Andrea Schroll,
  • Judith Löffler-Ragg,
  • Günter Weiss,
  • Thomas Sonnweber

摘要

Pulmonary arterial hypertension (PAH) is a progressive and life-limiting condition caused by pulmonary vascular remodeling due to heterogeneous aetiologies. Despite significant advances in treatment, PAH remains incurable and is associated with a poor prognosis, highlighting the need for novel therapeutic options. Recent advances in molecular and translational research have uncovered key pathways involved in PAH, including endothelial dysfunction, smooth muscle cell proliferation, inflammation, and thrombosis. These insights have guided the development of novel therapeutic strategies aimed at modulating these mechanisms. Promising compounds currently under investigation include agents targeting the bone morphogenic protein/transforming growth factor-beta axis, epigenetic modulators, receptor tyrosine kinase inhibitors, and immunomodulatory biologics. In addition, vasodilators acting on the renin–angiotensin–aldosterone system and the soluble guanylate cyclase pathway, as well as metabolic and hormonal modulators are under investigation, with clinical trials showing encouraging results for several agents. This review outlines the current understanding of PAH pathobiology and the most promising emerging treatments for pulmonary hypertension.