Pulmonary arterial hypertension (PAH) is a disorder in which mechanical obstruction of the
pulmonary vascular bed is largely responsible for the rise in mean pulmonary arterial
pressure, resulting in a progressive functional decline despite current available therapeutic
options. The fundamental pathogenetic mechanisms underlying this disorder include
pulmonary vasoconstriction, in situ thrombosis, medial hypertrophy, and intimal proliferation,
leading to occlusion of the small to mid-sized pulmonary arterioles and the formation of …