The DOI matches its Crossref record; 166 of the 166 references it cites were found in a registry or an index.
Basic Science of Pulmonary Arterial Hypertension for Clinicians: New Concepts and Experimental Therapies
Open accessAbstract
Pulmonary arterial hypertension (PAH) is a syndrome in which pulmonary arterial obstruction increases pulmonary vascular resistance, which leads to right ventricular (RV) failure and a 15% annual mortality rate. The present review highlights recent advances in the basic science of PAH. New concepts clarify the nature of PAH and provide molecular blueprints that explain how PAH is initiated and maintained. Five basic science concepts provide a framework to understand and treat PAH: (1) Endothelial dysfunction creates an imbalance that favors vasoconstriction, thrombosis, and mitogenesis. Restoration of this balance by inhibition of endothelin and thromboxane or augmentation of nitric oxide (NO) and prostacyclin is the paradigm on which most current therapy is based. (2) PAH has a genetic component. Mutations (bone morphogenetic protein receptor-2 [BMPR2]) and single-nucleotide polymorphisms (SNPs; ion channels and transporter genes) predispose to PAH. (3) Excess proliferation, impaired apoptosis, and glycolytic metabolism in pulmonary artery smooth muscle, fibroblasts, and endothelial cells suggest analogies to cancer. Many experimental therapies reduce PAH by decreasing the proliferation/apoptosis ratio; these include inhibitors of pyruvate dehydrogenase kinase (PDK), serotonin transporters (SERT), survivin, 3-hydroxy-3-methylglutaryl coenzyme A reductase, transcription factors (hypoxia-inducible factor [HIF]-1α and nuclear factor of activated T lymphocytes [NFAT]), and tyrosine kinases. Augmentation of voltage-gated K+ channels (Kv1.5) and BMPR2 signaling also addresses this imbalance. Tyrosine kinase inhibitors used to treat cancer are currently in phase 1 PAH trials. (4) Refractory vasoconstriction may occur due to rho kinase activation. Fewer than 20% of PAH patients respond to conventional vasodilators; however, refractory vasoconstriction may respond to rho kinase inhibitors. (5) The RV can be targeted therapeutically. Although increased afterload initiates RV failure, which is the major cause of death/dysfunction in PAH, the RV may be amenable to cardiac-targeted therapies. The RV in PAH has features of ischemic, hibernating myocardium. Guided by these new …
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Self-citation
Cited by
Pulmonary Arterial Hypertension: The Clinical Syndrome
Lai, Y.-C., Potoka, K. C., Champion, H. C., Mora, A. L., & Gladwin, M. T. (2014) · Circulation Research · doi:10.1161/circresaha.115.301146 · Cited by 402
The Metabolic Theory of Pulmonary Arterial Hypertension
Paulin, R., & Michelakis, E. D. (2014) · Circulation Research · doi:10.1161/circresaha.115.301130 · Cited by 314
Vita, J. A. (2011) · Circulation · doi:10.1161/circulationaha.111.078824 · Cited by 195
The nox4 inhibitor gkt137831 attenuates hypoxia-induced pulmonary vascular cell proliferation
Green, D. E., Murphy, T. C., Kang, B.-Y., Kleinhenz, J. M., Szyndralewiez, C., Page, P., Sutliff, R. L., & Hart, C. M. (2012) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2011-0418oc · Cited by 146
Baliga, R. S., Milsom, A. B., Ghosh, S. M., Trinder, S. L., MacAllister, R. J., Ahluwalia, A., & Hobbs, A. J. (2012) · Circulation · doi:10.1161/circulationaha.112.100586 · Cited by 110
Metadata
- Authors
- Archer, S. L., Weir, E. K., & Wilkins, M. R.
- Venue
- Circulation
- Published
- 2010-05-11
- Volume
- 121
- Issue
- 18
- Pages
- 2045-2066
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2065501812
- Cited by
- 485
- References
- 166
- FWCI
- 36.40
- Type
- journal article
- Language
- en
- Open access
- green
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
