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 dysregulated 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.
Bibliographical noteFunding Information:
Dr. Prisco is funded by National Institutes of Health (NIH) grant T32 HL144472, a University of Minnesota Clinical and Translational Science award (NIH UL1 TR0029494), and a University of Minnesota Medical School Academic Investment Educational Program grant. Dr. Prins is funded by NIH K08 HL140100, the Jenesis Award from United Therapeutics, a Lillehei Heart Institute Cardiovascular Seed Grant, and the Cardiovascular Medical Research and Education Fund; and has served as a consultant for Actelion and receives grant funding from United Therapeutics. Dr. Thenappan has served as a consultant for Actelion and Gilead. The content of this article is solely the responsibility of the authors and does not represent the official views of the NIH or any other funding sources. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
© 2020 The Authors
- clinical trials
- pulmonary arterial hypertension
- right ventricle
PubMed: MeSH publication types
- Journal Article