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Editorial
. 2008 Jan;3(1):1-4.
doi: 10.4103/1817-1737.37832.

Pulmonary hypertension: past, present and future

Robyn J Barst
Editorial

Pulmonary hypertension: past, present and future

Robyn J Barst. Ann Thorac Med. 2008 Jan.
No abstract available

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Figures

Figure 1
Figure 1
Targeted medical therapy for pulmonary arterial hypertension based on the prostacyclin pathway, the nitric oxide pathway and the endothelin pathway. Reprinted with permission from Humbert et al., N Engl J Med 2004
Figure 2
Figure 2
Some cellular processes implicated in the pathogenesis of PAH. Extracellular mediators and cells (platelets) are highlighted in yellow, cell surface receptors and ion channels in purple, intracellular signaling in blue and nuclear responses in green. See text for detailed descriptions of pathogenic mechanisms and interactions among the many pathways that span the extracellular, membrane, cytosolic and nuclear domains. VEGF indicates vascular endothelial growth factor; its receptor is KDR. Intracellular transduction of this pathway is poorly understood. Endothelin is vasoactive and a mitogen, acting through Ca2 + channels and ERK/Jun kinases. Tyrosine kinase is the angiopoietin receptor, a system found to be upregulated in pulmonary vascular disease.56 Alk 1 and BMPR1-2 are receptors of the TGF-β superfamily, and BMP indicates bone morphogenetic protein. Alk 1 mutations cause hereditary hemorrhagic telangiectasia and some cases of Idiopathic Pulmonary Arterial Hypertension. Epidermal growth factor (EGF), tumour necrosis factor (TNF)-, angiotensin II (ANGII) and platelet-derived growth factor (PDGF) are all proliferative stimuli that act through tyrosine kinase receptors and are partially transduced by intracellular oxidant species. In the intracellular domain, SMADs are regulatory proteins that activate nuclear transcription factors and interact with MAP kinases. AML 1 is a nuclear transcription factor of potential importance. Elastase, downstream of AML 1, has been implicated in vascular disease in experimental animals. Viral proteins are found in vascular lesions in the lungs of patients with PAH, raising the possibility that they participate in its pathogenesis. Reprinted with permission from
Figure 3
Figure 3
Pulmonary arterial hypertension: a historical perspective
Figure 4
Figure 4
Current consensus evidence-based guidelines for the treatment of pulmonary arterial hypertension
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