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J. Clin. Med. 2017, 6(4), 43; doi:10.3390/jcm6040043

Emerging Metabolic Therapies in Pulmonary Arterial Hypertension

1
Medical Scientist Training Program, University of Pittsburgh School of Medicine and University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
2
Division of Cardiology, Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung, Blood, and Vascular Medicine Institute, University of Pittsburgh School of Medicine and University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
*
Author to whom correspondence should be addressed.
Academic Editor: Jane A. Leopold
Received: 15 February 2017 / Revised: 28 March 2017 / Accepted: 29 March 2017 / Published: 4 April 2017
(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Arterial Hypertension)
View Full-Text   |   Download PDF [1627 KB, uploaded 5 April 2017]   |  

Abstract

Pulmonary hypertension (PH) is an enigmatic vascular disorder characterized by pulmonary vascular remodeling and increased pulmonary vascular resistance, ultimately resulting in pressure overload, dysfunction, and failure of the right ventricle. Current medications for PH do not reverse or prevent disease progression, and current diagnostic strategies are suboptimal for detecting early-stage disease. Thus, there is a substantial need to develop new diagnostics and therapies that target the molecular origins of PH. Emerging investigations have defined metabolic aberrations as fundamental and early components of disease manifestation in both pulmonary vasculature and the right ventricle. As such, the elucidation of metabolic dysregulation in pulmonary hypertension allows for greater therapeutic insight into preventing, halting, or even reversing disease progression. This review will aim to discuss (1) the reprogramming and dysregulation of metabolic pathways in pulmonary hypertension; (2) the emerging therapeutic interventions targeting these metabolic pathways; and (3) further innovation needed to overcome barriers in the treatment of this devastating disease. View Full-Text
Keywords: pulmonary arterial hypertension; metabolism; metabolic reprogramming; hypoxia-inducible factor; therapeutics pulmonary arterial hypertension; metabolism; metabolic reprogramming; hypoxia-inducible factor; therapeutics
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Harvey, L.D.; Chan, S.Y. Emerging Metabolic Therapies in Pulmonary Arterial Hypertension. J. Clin. Med. 2017, 6, 43.

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