Pulmonary arterial hypertension (PAH) is a progressive disease of the lung vasculature, which is characterized by sustained pulmonary arterial pressure, resulting in increased pulmonary vascular resistance, with eventual right heart failure . Vascular remodeling caused by the medial hyperplasia of pulmonary artery (PA) smooth muscle cells is a hallmark feature of PAH , which causes occlusion of the vessels . In most forms of PAH, muscularization of small distal PA occurs , and is further characterized by excessive vascular cell proliferation, inward remodeling, rarefaction, and a loss of compliance of the pulmonary blood vessels . Increased resistance to blood flow and more rigid blood vessels (loss of vascular compliance) leads to failure of the right ventricle and eventual death. PAH is more frequent in women than men, and left untreated has a survival time of 5–7 years post diagnosis . From a therapeutic standpoint, there are a number of vasodilator drugs that are indicated for the treatment of PAH, but none of the current therapeutics offers long-term success for survival due to limited effectiveness and unwanted side effects , and more importantly, do not address the underlying causes of the disease.