Pulmonary hypertension (PH) is a disease marked by a combination of constriction and remodeling within the pulmonary vasculature. It remains a disease without a cure, as current treatments were developed with a focus on vasodilatory properties but do not reverse the remodeling component. Numerous recent advances have been made in the understanding of cellular processes that drive pathologic remodeling in each layer of the vessel wall as well as the accompanying maladaptive changes in the right ventricle. In particular, the past few years have yielded much improved insight into the pathways that contribute to altered metabolism, mitochondrial function, and reactive oxygen species signaling and how these pathways promote the pro-proliferative, pro-migratory, and anti-apoptotic phenotype of the PH vasculature. Additionally, there have been significant advances in numerous other pathways linked to disease pathogenesis, such as sex hormones and perivascular inflammation. Novel insights into cellular pathology have suggested new avenues for the development of both biomarkers and therapies that will hopefully bring us closer to the elusive goal: a therapy leading to reversal of disease.
- pulmonary arterial hypertension
- Copyright © 2016, American Journal of Physiology-Lung Cellular and Molecular Physiology