Advance in the basic research on pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is defined as a group of diseases characterized by a rapidly increased pulmonary arterial pressure and pulmonary vascular resistance. The pathology of PAH includes vasoconstriction, vascular wall remodeling, hyperplasia of pulmonary artery smooth muscle cells and pulmonary endothelial cells, in situ thrombosis. The etiology of PAH is complex, multifactorial, and likely involves the genetic predisposition such as mutations in bone morphogenetic protein receptor-II and activin receptor-like kinase 1. In addition, the down-regulation of peroxisome proliferator-activated receptors activity, over-expression of RhoA / Rho kinase may play a critical role in pathophysiology of PAH. This finding will provide more theoretical basis on prevention and treatment of this disease.