Pulmonary arterial hypertension: New insights and new hope

Abstract

Pulmonary arterial hypertension (PAH) is a devastating disorder characterized by abnormal increased vasoconstriction and vascular remodelling. In this review we discuss the pathophysiology, genetic basis and clinical features of this disorder. Current therapy of PAH is based on an understanding of its pathogenesis, and we review current treatment options based on the pathophysiology of the disease. We discuss three promising novel therapies studied in animal models and human tissue. All three therapies appear to prevent and reduce pulmonary arterial medial hyperplasia through their anti-proliferative and/or pro-apoptotic effects: serotonin transporter inhibitors by blocking serotonin uptake; dichloroacetate by activating voltage-gated potassium channels; and simvastatin by preventing activation of small GTPases.