Open AccessNew and Emerging Therapies for Pulmonary Arterial Hypertension
Author(s) -
Edda Spiekerkoetter,
Steven M. Kawut,
Vinicio A. de Jesus Perez
Publication year - 2019
Publication title -
annual review of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.03
H-Index - 160
eISSN - 1545-326X
pISSN - 0066-4219
DOI - 10.1146/annurev-med-041717-085955
Subject(s) - medicine , pulmonary hypertension , bioinformatics , clinical trial , translational research , hormone , cardiology , intensive care medicine , pathology , biology
Pulmonary arterial hypertension (PAH) is a pulmonary vasculopathy that causes right ventricular dysfunction and exercise limitation and progresses to death. New findings from translational studies have suggested alternative pathways for treatment. These avenues include sex hormones, genetic abnormalities and DNA damage, elastase inhibition, metabolic dysfunction, cellular therapies, and anti-inflammatory approaches. Both novel and repurposed compounds with rationale from preclinical experimental models and human cells are now in clinical trials in patients with PAH. Findings from these studies will elucidate the pathobiology of PAH and may result in clinically important improvements in outcome.