title: Current treatment strategies for pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension (PAH) is a disease characterized by an elevation in pulmonary artery pressure that can lead to right ventricular failure and death. Although there is no cure for PAH, newer medical therapies have been shown to improve a variety of clinically relevant end-points including survival, exercise tolerance, functional class, haemodynamics, echocardiographic parameters and quality of life measures. Since the introduction of continuous intravenous prostacyclin, the treatment armamentarium of approved drugs for PAH has expanded to include prostacyclin analogues with differing routes of administration, a dual endothelin receptor antagonist, and a phosphodiesterase-5 inhibitor. Selective endothelin-A receptor antagonists have shown promise in clinical trials and are likely to be added to the list of options. As the number of medications available for PAH continues to increase, treatment decisions regarding first-line therapy, combination treatments, and add-on strategies are becoming more complex. This article reviews the current treatments strategies for PAH and provides guidelines for its management.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of California, San Diego, La Jolla, CA 92037-7381, USA.
authors.forename: S H
authors.initials: SH
authors.lastname: Lee
authors.forename: L J
authors.initials: LJ
authors.lastname: Rubin
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D065130:Endothelin A Receptor Antagonists; D065131:Endothelin B Receptor Antagonists; D016041:Heart-Lung Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy*; D010102:Oxygen Inhalation Therapy; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D017410:Practice Guidelines as Topic; D011453:Prostaglandins / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D065130:Endothelin A Receptor Antagonists; D065131:Endothelin B Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins