title: Management of pulmonary arterial hypertension.
abstract: OBJECTIVE
  Pulmonary arterial hypertension (PAH) is a complex disease with a high mortality. Management of this disease is underpinned by supportive and general therapies delivered by multidisciplinary teams in specialist centres. In recent years, a number of PAH-specific therapies have improved patient outcomes. This article will discuss the management of PAH in the context of relevant recently published studies in this area.
  
  
  RESULTS
  PAH-specific therapies are targeted towards dysfunctional signalling identified within the pulmonary circulation, and include endothelin receptor antagonists, phosphodiesterase type-5 inhibitors and prostanoids. Combination of these therapies is considered in patients with more severe disease. In addition, timely referral for surgical intervention (e.g. atrial septostomy, lung transplantation) should be made in selected patients with advanced disease. New treatment modalities currently in development may further improve patient outcomes in future years. However, further development and expansion of patient registries is vital for enhanced understanding of this disease, and may guide the optimal use of existing therapies and the development of new treatment approaches.
  
  
  CONCLUSIONS
  Outcomes in PAH have improved in recent years through a management approach characterized by general and supportive measures, and PAH-specific and surgical therapies in selected patients. Continued development of patient registries is vital to improve understanding and outcomes of this disease.
authors.affiliation: National Pulmonary Hypertension Unit, Department of Respiratory Medicine, Mater Misericordiae University Hospital, Dublin, Ireland.
authors.forename: Eoin P
authors.initials: EP
authors.lastname: Judge
authors.forename: Sean P
authors.initials: SP
authors.lastname: Gaine
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use*; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D004232:Diuretics / Q000627:therapeutic use*; D065128:Endothelin Receptor Antagonists; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D017408:Guidelines as Topic; D006801:Humans; D006931:Hyperbaric Oxygenation; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality / Q000601:surgery; D008297:Male; D017063:Outcome Assessment, Health Care; D018579:Patient Selection; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D011453:Prostaglandins / Q000627:therapeutic use*; D012042:Registries
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D004232:Diuretics; D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D011453:Prostaglandins