The Shared Pathogenesis of Pulmonary Artery Hypertension

Abstract

Pulmonary artery hypertension is defined as an increased in pulmonary artery pressure
exceeding 25 mmHg with normal pulmonary wedge pressure. The pathogenesis of pulmonary
artery hypertension involves interaction among vascular, cellular and biomarker components
in the pulmonary tissue; with eventual result is elevated pulmonary artery pressure. Vascular
components are remodeling of intimal, medial and adventitial layers. Cellular components are
played by apoptosis-resistant endothelial cells, proliferative-prone pulmonary artery smooth
muscle cells, fibroblasts and inflammatory cells. The functional biomarkers are produced and
mediated by these cellular changes, mainly endothelin-1, thromboxane, serotonin, nitric oxide,
and prostacyclin. The pulmonary vascular remodeling in pulmonary artery hypertension are
diverse and may present in various severity based on underlying etiology. Understanding the
shared pathogenesis in pulmonary artery hypertension is of paramount importance in order to
improve the disease management and treatment approach.