title: Current therapeutics and practical management strategies for pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension (PAH) develops from an abnormal interaction between the endothelium and smooth muscle cells in the pulmonary vasculature and is characterized by a progressive rise in pulmonary vascular resistance resulting from vascular remodeling, vasoconstriction, and cellular proliferation. Currently, 3 classes of drugs are approved for the treatment of PAH based on results from small short-term clinical trials-prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors. The pharmacologic management of PAH is rapidly evolving as newer therapeutic targets that stabilize or reverse pulmonary vascular disease and target right ventricular function are being sought and as clinical practice patterns shift in favor of earlier diagnosis and aggressive treatment. This manuscript will review the practical management aspects of currently approved PAH treatments and briefly discuss combination therapy and novel pharmacologic targets. In addition, the treatment of acute right ventricular failure and evidence (or lack thereof) for therapies in non-PAH pulmonary hypertension, such as pulmonary hypertension from left side of the heart disease, are addressed.
authors.affiliation: Section of Cardiology, Department of Medicine, University of Chicago, IL, USA.
authors.forename: Richa
authors.initials: R
authors.lastname: Agarwal
authors.forename: Mardi
authors.initials: M
authors.lastname: Gomberg-Maitland
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D065128:Endothelin Receptor Antagonists; D004730:Endothelium, Vascular / Q000187:drug effects / Q000503:physiopathology; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology; D009017:Morbidity / Q000639:trends; D009131:Muscle, Smooth, Vascular / Q000187:drug effects / Q000503:physiopathology; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use*; D017410:Practice Guidelines as Topic; D011379:Prognosis; D011651:Pulmonary Artery / Q000187:drug effects / Q000503:physiopathology*; D014481:United States / Q000453:epidemiology; D014655:Vascular Resistance / Q000187:drug effects / Q000502:physiology*; D014661:Vasoconstriction / Q000187:drug effects; D016278:Ventricular Function, Right / Q000187:drug effects / Q000502:physiology*
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors