ABSTRACT

Pulmonary arterial hypertension (PAH) is a disease of the small pulmonary arteries, characterized by vascular proliferation and remodeling (1,2). It leads to progressive increase in pulmonary vascular resistance (PVR), ultimately causing right ventricular failure and death. Despite recent major improvements, no current treatments of PAH are curative for this devastating condition. However, in less than 20 years, treatment for patients with PAH has evolved from a state of no hope to one in which prolonged survival and improvements in quality of life can be achieved (1).