ABSTRACT
Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure (mPAP) greater than 25 mmHg in the setting of normal or reduced cardiac output (CO) and normal pulmonary capillary wedge pressure (PCWP) (15 mmHg) (1). PAH is a disease of the small pulmonary arteries characterized by vascular proliferation and remodeling (2). It results in a progressive increase in pulmonary vascular resistance (PVR), right ventricular failure, and ultimately death. PAH can be characterized as idiopathic (IPAH) or familial, or may be associated with other conditions including collagen vascular disease, congenital heart disease (CHD), portal hypertension, HIV infection, and the use of certain drugs (3). PAH most commonly occurs in women of childbearing age (4). During the last decade, new advanced therapies for the treatment of PAH have evolved, improving the quality of life and outcomes of PAH patients. However, despite these recent therapeutic advances, no treatment can cure this devastating condition (5).
