Pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a debilitating disease characterized by progressive adverse remodeling of the resistance pulmonary arteries, ultimately leading to right ventricular (RV) failure and death. It is defined by increases in pulmonary arterial pressures (PAP), pulmonary vascular resistance (PVR), and ultimately, right ventricular failure. The field of PAH has made remarkable progress in the last two decades with understanding in the pathogenesis and improvements in therapeutic and prognostic tools. In this chapter, we will review the definition, pathogenesis, epidemiology, clinical presentation, management, and prognostication of PAH in the current era.