Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 May;83(7):577-585.
doi: 10.1007/s40265-023-01862-z. Epub 2023 Apr 5.

Novel Drugs for the Treatment of Pulmonary Arterial Hypertension: Where Are We Going?

Ayedh K Alamri  1   2   3 , Christy L Ma  4 , John J Ryan  4
Affiliations

Novel Drugs for the Treatment of Pulmonary Arterial Hypertension: Where Are We Going?

Ayedh K Alamri et al. Drugs. 2023 May.

Abstract

Pulmonary arterial hypertension (PAH) is a progressive disease that despite advances in therapy is associated with a 7-year survival of approximately 50%. Several risk factors are associated with developing PAH, include methamphetamine use, scleroderma, human immunodeficiency virus, portal hypertension, and genetic predisposition. PAH can also be idiopathic. There are traditional pathways underlying the pathophysiology of PAH involving nitric oxide, prostacyclin, thromboxane A2, and endothelin-1, resulting in impaired vasodilation, enhanced vasoconstriction and proliferation in the pulmonary vasculature. Established PAH medications targets these pathways; however, this paper aims to discuss novel drugs for treating PAH by targeting new and alternative pathways.

PubMed Disclaimer

Conflict of interest statement

Ayedh K. Alamri, Christy L. Ma, and John J. Ryan declare they have no potential conflicts of interest that might be relevant to the contents of this manuscript.

Figures

Fig. 1
Fig. 1
Current treatments for pulmonary arterial hypertension. The pathophysiology and current therapeutic targets of pulmonary arterial hypertension (adapted from the 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension [15]). NO nitric oxide, PDE5 phosphodiesterase 5, sGC soluble guanylate cyclase, cGMP (cyclic) guanosine monophosphate, GTP guanosine-5′-triphosphate, IP receptor prostacyclin I2 receptor, cAMP cyclic adenosine monophosphate, ESC European Society of Cardiology, ERS European Respiratory Society
Fig. 2
Fig. 2
Novel pathways in treating pulmonary arterial hypertension. The figure shows alternative pathways discussed in this paper in treating PAH. Various mechanisms contribute to the development of PAH, such as inflammation, vasoconstriction, abnormal endothelial and smooth muscle cell growth, proliferation, and apoptosis (created with BioRender). BMP bone morphogenetic protein, BMPR-II BMP receptor type 2, EC endothelial cells, PDGF platelet-derived growth factor, PDGFR PDGF receptor, SMC smooth muscle cells, TGF-β transforming growth factor-β, TPH1 tryptophan hydroxylase 1, VIP vasoactive intestinal peptide, VPAC vasoactive intestinal peptide receptor
See this image and copyright information in PMC

References

    1. Alamri AK, Ma CL, Ryan JJ. Left heart disease-related pulmonary hypertension. Cardiol Clin. 2022;40(1):69–76. doi: 10.1016/j.ccl.2021.08.007. - DOI - PubMed
    1. Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. doi: 10.1183/13993003.01913-2018. - DOI - PMC - PubMed
    1. Sommer N, Ghofrani HA, Pak O, Bonnet S, Provencher S, Sitbon O, et al. Current and future treatments of pulmonary arterial hypertension. Br J Pharmacol. 2021;178(1):6–30. doi: 10.1111/bph.15016. - DOI - PubMed
    1. Gall H, Felix JF, Schneck FK, Milger K, Sommer N, Voswinckel R, et al. The Giessen Pulmonary Hypertension Registry: survival in pulmonary hypertension subgroups. J Heart Lung Transplant. 2017;36(9):957–967. doi: 10.1016/j.healun.2017.02.016. - DOI - PubMed
    1. Lan NS, Massam BD, Kulkarni SS, Lang CC. Pulmonary arterial hypertension: pathophysiology and treatment. Diseases. 2018;6(2):38. doi: 10.3390/diseases6020038. - DOI - PMC - PubMed

MeSH terms