Updates on the Treatment of Pulmonary Hypertension

Lee, Sang Eun; Chang, Hyuk Jae

Abstract

Pulmonary arterial hypertension (PAH) is a rare and severe clinical condition characterized by luminal obliteration of small pulmonary arteries. It results in increased resistance to pulmonary blood flow, and ultimately, right ventricular failure and death. Although no curative treatments exists for PAH, improved understanding of the pathobiological mechanisms of this disease has resulted in the development of effective therapies that target specific aberrant pathways during the past 20 years. Current treatments including prostanoids, endothelin-1 (ET-1) antagonists, and phosphodiesterase (PDE) inhibitors, have been demonstrated in randomized, controlled studies to confer improvements in functional status, pulmonary hemodynamics, and possibly even slow disease progression. Several alternative pathways believed to play an important role in the pathogenesis of PAH have been identified as potentially useful therapeutic targets and a number of investigative approaches focusing on these targets are under active development. As the number of options available for PAH treatment continues to increase, treatment decisions regarding first-line therapy, combination treatments, and add-on strategies are becoming more complex. This review article focuses on pharmacological agents currently available for the treatment of PAH and discusses potential novel option of treatment strategies.