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Review
. 2025 Feb 10:19:e18743064344024.
doi: 10.2174/0118743064344024250203101417. eCollection 2025.

Updates on Pulmonary Hypertension

Vivek Paudyal  1 , Rubi Thapa  1 , Sagarika Basnet  2 , Munish Sharma  3 , Salim Surani  4 , Joseph Varon  5
Affiliations
Review

Updates on Pulmonary Hypertension

Vivek Paudyal et al. Open Respir Med J. .

Abstract

Pulmonary Arterial Hypertension (PAH) is an uncommon condition with high mortality. It is an underrecognized condition both in developing and developed countries, especially in developing countries, due to a lack of advanced healthcare facilities and resources for timely diagnosis. More than half of the individuals diagnosed with PAH live less than five years after diagnosis. In recent years, tremendous advancements have been made in diagnostic and therapeutic strategies for PAH patients. Phosphodiesterase 5 (PDE5) inhibitors, endothelin receptor antagonists, and prostacyclin inhibitors in various forms (oral, inhaled, intravenous, or subcutaneous) have been the cornerstone of medical treatment. Atrial septostomy, heart and lung transplant, balloon pulmonary angioplasty, and pulmonary thromboendarterectomy are existing therapeutic options currently available. There has been a continuous effort to introduce newer therapies to improve life expectancy and modify disease. Newer therapies have shown promising results but require future data to guarantee long-term safety and efficacy. We aim to discuss a few of these critical updates in the constantly evolving field of PAH.

Keywords: Inhaled Treprostinil; Macitentan; Pulmonary hypertension; Remote pulmonary artery pressure monitoring; Sotatercept; Tadalafil.

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Conflict of interest statement

Dr. Joseph Varon is Editor in Chief, Dr. Salim R. Surani is Co-editor in Chief, Dr. Munish Sharma is the Editorial Board Member of The Open Respiratory Medicine Journal.

Figures

Fig. (1)
Fig. (1)
A) Figure showing the balance between Activin A and BMP. B) The figure shows the pro-proliferative action of Activin A due to a mutation in the BMP gene.
Fig. (2)
Fig. (2)
Adverse events occurring at a higher frequency in the Sotatercept group.
Fig. (3)
Fig. (3)
Pathophysiology of PAH, including the targeted site of action of Macitentan and Tadalafil [1, 3]. cAMP: cyclic Adenosine Monophosphate, cGMP: cyclic Guanosine Monophosphate, ETA: Endothelin A receptor, ETB: Endothelin B receptor, NO: Nitric Oxide, PDE5: Phosphodiesterase 5, PGI2: Prostacyclin.
Fig. (4)
Fig. (4)
Adverse effects in patients treated with M/T FDC during the 16-week double-blind study portion of A DUE study [35]; modifications in Images by Goran tek-en (CC-BY-SA 4.0), Mikael Haggstrom (CC0).
Fig. (5)
Fig. (5)
Pathogenesis of ILD complicated by PH along with the mechanism of action of Treprostinil. CO2: Carbon dioxide, ET-1: Endothelin 1, NO: Nitric Oxide, O2: Oxygen, PDGF: Platelet Derived Growth Factor, PGI2: Prostacyclin, VEGF: Vascular Endothelial Growth Factor.
Fig. (6)
Fig. (6)
Adverse effects following administration of Treprostinil via different routes [44].
See this image and copyright information in PMC

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