microRNA and Pulmonary Hypertension.

Pulmonary arterial hypertension (PAH) is a lethal vasculopathy associated with complex etiology that involves remodeling of distal pulmonary arteries leading to elevation of pulmonary vascular resistance. This process results in right ventricular (RV) hypertrophy and ultimately RV failure. In addition, PAH is associated with systemic impairment in the skeletal muscle contributing to exercise intolerance. It has only been a few decades since microRNAs (miRNAs) have been implied in the development and progression of PAH regarding every organ affected by the disease. Indeed, impairment of miRNA's expression has been involved in vascular cell remodeling processes such as adventitial fibroblast (AdvFB) migration; pulmonary arterial smooth muscle cell (PASMC) proliferation and pulmonary arterial endothelial cell (PAEC) dysfunction observed in PAH. At the molecular level miRNAs have been described in the control of ion channels and mitochondrial function as well as the regulation of the BMPR2 signaling pathways contributing to PAH lung impairment. Recently miRNAs have also been specifically implicated in RV dysfunction and systemic angiogenic impairment, observed in PAH. In this chapter, we will summarize the knowledge on miRNA in PAH and highlight their crucial role in the etiology of this disease.