4.6 Review

Treatment Targets for Right Ventricular Dysfunction in Pulmonary Arterial Hypertension

Journal

JACC-BASIC TO TRANSLATIONAL SCIENCE
Volume 5, Issue 12, Pages 1244-1260

Publisher

ELSEVIER SCIENCE INC
DOI: 10.1016/j.jacbts.2020.07.011

Keywords

clinical trials; pulmonary arterial hypertension; right ventricle

Funding

  1. National Institutes of Health (NIH) [T32 HL144472]
  2. University of Minnesota Clinical and Translational Science award [NIH UL1 TR0029494]
  3. University of Minnesota Medical School Academic Investment Educational Program grant
  4. NIH [K08 HL140100]
  5. Jenesis Award from United Therapeutics
  6. Cardiovascular Medical Research and Education Fund
  7. United Therapeutics
  8. Lillehei Heart Institute Cardiovascular Seed Grant

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Right ventricle (RV) dysfunction is the strongest predictor of mortality in pulmonary arterial hypertension (PAH), but, at present, there are no therapies directly targeting the failing RV. Although there are shared molecular mechanisms in both RV and left ventricle (LV) dysfunction, there are important differences between the 2 ventricles that may allow for the development of RV-enhancing or RV-directed therapies. In this review, we discuss the current understandings of the dysregutated pathways that promote RV dysfunction, highlight RV-enriched or RV-specific pathways that may be of particular therapeutic value, and summarize recent and ongoing clinical trials that are investigating RV function in PAH. It is hoped that development of RV-targeted therapies will improve quality of life and enhance survival for this deadly disease. (C) 2020 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation.

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