Pathways in pulmonary arterial hypertension: the future is here
Open accessAbstract
It is well established that the endothelin, nitric oxide and prostacyclin pathways play an important role in the development of pulmonary arterial hypertension (PAH). Indeed, the therapeutic options currently available for the management of PAH all act on one of these mechanistic pathways. However, this is an exciting time for both clinicians and scientists, as increased understanding of the mechanisms involved in the pathogenesis and progression of PAH has resulted in the development of a number of novel therapeutic options. This article highlights how the introduction of new compounds such as macitentan, riociguat and selexipag, which act on the endothelin, nitric oxide and prostacyclin pathways, respectively, have the potential to further improve the prognosis for patients with PAH.
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All types of PH develop via an early, common vasculopathy driven by disproportionate pulmonary vasoconstriction and dramatic remodeling of the endothelium and vessel wall.
“Pathologically, all types of PH develop via an early, common vasculopathy driven by disproportionate pulmonary vasoconstriction and dramatic remodeling of the endothelium and vessel wall[5,26,27].”
From Enterosalivary nitrate metabolism and the microbiome: Intersection of microbial metabolism, nitric oxide and diet in cardiac and pulmonary vascular health (2017) · cites this paper as [27]
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- Authors
- Sitbon, O., & Morrell, N.
- Affiliations
- Université Paris-Sud (FR)
- ORCID
- Sitbon, Olivier
- Venue
- European Respiratory Review
- Publisher
- European Respiratory Society (ERS)
- Published
- 2012-11-30
- Volume
- 21
- Issue
- 126
- Pages
- 321-327
- ISSN
- 0905-9180 (ISSN-L)
- 1600-0617
- PMC
- PMC9487224
- OpenAlex
- W2163323710
- Cited by
- 117
- References
- 59
- Citations by year
- 2026: 3 · 2024: 6 · 2023: 2 · 2022: 7 · 2021: 14 · 2020: 3 · 2019: 8 · 2018: 10 · 2017: 8 · 2016: 12 · 2015: 10 · 2014: 21 · 2013: 11 · 2012: 1
- FWCI
- 2.32
- Type
- journal article
- Language
- en
- Open access
- diamond
- doi.org
- Locations
- European Respiratory Review (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- Actelion Pharmaceuticals
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Genetics › Vascular Anomalies and Treatments
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Keywords
- pulmonary arterial hypertension, endothelin pathway, nitric oxide pathway, prostacyclin pathway, macitentan, riociguat, selexipag, PAH therapy, endothelin receptor antagonists, soluble guanylate cyclase stimulator, prostacyclin analogues
- MeSH terms
- Imatinib Mesylate, Acetamides, Animals, Antihypertensive Agents, Benzamides, Clinical Trials as Topic, Guanylate Cyclase, Humans, Hypertension, Pulmonary, Lisuride, Nitric Oxide, Piperazines, Epoprostenol, Pyrazines, Pyrazoles, Pyrimidines, Serotonin Antagonists, Sulfonamides, Drugs, Investigational, Endothelin-1, Protein Kinase Inhibitors, Phosphodiesterase 5 Inhibitors, Endothelin A Receptor Antagonists, Endothelin B Receptor Antagonists
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