Abstract
Pulmonary hypertension (PH) is an often-fatal vascular disease of unclear molecular origins. The pulmonary vascular remodelling which occurs in PH is characterised by elevated vasomotor tone and a pro-proliferative state, ultimately leading to right ventricular dysfunction and heart failure. Guided in many respects by prior evidence from cancer biology, recent investigations have identified metabolic aberrations as crucial components of the disease process in both the pulmonary vessels and the right ventricle. Given the need for improved diagnostic and therapeutic options for PH, the development or repurposing of metabolic tracers and medications could provide an effective avenue for preventing or even reversing disease progression. In this review, we describe the metabolic mechanisms that are known to be dysregulated in PH; we explore the advancing diagnostic testing and imaging modalities that are being developed to improve diagnostic capability for this disease; and we discuss emerging drugs for PH which target these metabolic pathways.
Abstract
Understanding metabolic pathways in PH provides opportunities for improved diagnostic and therapeutic options http://ow.ly/pFQb30guez6
Footnotes
This article has been revised according to the correction published in the March 2018 issue of the European Respiratory Review.
Support statement: This work was supported by National Institutes of Health grants HL096834, HL124021, HL138437 and TR002073, and the American Heart Association grant 14GRNT19600012 (to S.Y. Chan). Funding was also received from Actelion Pharmaceuticals Ltd, Allschwil, Switzerland (to L.J. Rubin). Funding information for this article has been deposited with the Crossref Funder Registry.
Conflict of interest: Disclosures can be found alongside this article at err.ersjournals.com
Provenance: Publication of this peer-reviewed article was sponsored by Actelion Pharmaceuticals Ltd, Allschwil, Switzerland (principal sponsor, European Respiratory Review issue 146).
- Received August 22, 2017.
- Accepted October 28, 2017.
- Copyright ©ERS 2017.
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