Pulmonary Arterial Hypertension: Diagnosis and Treatment

Abstract

Pulmonary arterial hypertension (PAH) is a specific, rare disease characterized by a well-described pattern of pulmonary vascular remodeling. The elevated pulmonary artery pressure in PAH results in increased right ventricular afterload, which, if untreated, leads rapidly to right ventricular failure and death. Recent marked expansion in knowledge about PAH has resulted in the development of effective therapies that improve quality of life and survival. However, delays in diagnosis and suboptimal treatment remain significant barriers to achieving optimal patient outcomes. Continued success in raising PAH awareness, earlier diagnosis, and the availability of new therapies mean a promising future for PAH patients.

Keywords: Echocardiography; Endothelin antagonist; Phosphodiesterase inhibitor; Prostacyclin; Pulmonary arterial hypertension; Right heart catheterization.