Pulmonary Arterial Hypertension: New Pathophysiological Insights and Emerging Therapeutic Targets

Pulmonary arterial hypertension (PAH) encompasses a group of clinical entities characterized by sustained vasoconstriction and progressive vascular remodeling that act in concert to elevate pulmonary vascular resistance. The current treatments for PAH are mainly dedicated to target the process of vasoconstriction and do not offer a cure. There is now accumulating evidence that expansion of pulmonary artery cells due to increased proliferation and apoptotic evasion is a key pathological component of vascular remodeling that occurs in PAH. Thus, vascular lesions seen in advanced PAH patients present some cancer-like characteristics offering important avenues for exploration and expanding treatment options. In this review article, we will discuss recent advances into mechanisms underlying disease progression, with a focus on pulmonary artery smooth muscle cells.