New Therapeutic Pathways in Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a serious and progressive vascular disease characterized by arterial vasoconstriction, remodeling, and inflammation. Despite considerable progress in the treatment of patients with PAH, long-term survival remains poor. Currently approved therapies target three well-characterized pathways involved in pulmonary vascular tone, with a poorly understood effect, if any, on vascular remodeling. Over the last two decades, novel pathways implicated in PAH remodeling and pathogenesis have been unraveled. Such pathways involve uncontrolled cellular proliferation, excessive inflammation, metabolic abnormalities, mitochondrial dysfunction, and genetic dysregulation. Several pre-clinical studies and clinical trials are currently exploring the potential benefits and safety of new therapeutic agents targeting these pathways, with the aim of modifying the disease course and improving long-term survival.