title: Health-related quality of life in patients with pulmonary arterial hypertension.
abstract: BACKGROUND
  Improved outcomes with expanding treatment options for patients with pulmonary arterial hypertension present the opportunity to consider additional end-points in approaching therapy, including factors that influence health-related quality of life. However, comparatively little is known about health-related quality of life and its determinants in patients with pulmonary arterial hypertension.
  
  
  METHODS
  Health-related quality of life was evaluated in a cross sectional study of 155 outpatients with pulmonary arterial hypertension using generic and respiratory-disease specific measurement tools. Most patients had either World Health Organization functional Class II or III symptoms. Demographic, hemodynamic and treatment variables were assessed for association with health-related quality of life scores.
  
  
  RESULTS
  Patients with pulmonary arterial hypertension suffered severe impairments in both physical and emotional domains of health-related quality of life. Patients with idiopathic ("primary") pulmonary arterial hypertension had the best, and those with systemic sclerosis the worst health-related quality of life. Greater six-minute walk distance correlated with better health-related quality of life scores, as did functional Class II versus Class III symptoms. Hemodynamic measurements, however, did not correlate with health-related quality of life scores. No differences in health-related quality of life were found between patients who were being treated with calcium channel antagonists, bosentan or continuously infused epoprostenol at the time of quality of life assessment.
  
  
  CONCLUSIONS
  Health-related quality of life is severely impaired in patients with pulmonary arterial hypertension and is associated with measures of functional status. Specific associations with impaired health-related quality of life suggest potential areas for targeted intervention.
authors.affiliation: Pulmonary, Allergy and Critical Care Division, University of Pennsylvania School of Medicine, University of Pennsylvania Medical Center-Presbyterian, Philadelphia, PA 19104, USA. darren.taichman@uphs.upenn.edu
authors.forename: Darren B
authors.initials: DB
authors.lastname: Taichman
authors.forename: Jennifer
authors.initials: J
authors.lastname: Shin
authors.forename: Laryssa
authors.initials: L
authors.lastname: Hud
authors.forename: Christine
authors.initials: C
authors.lastname: Archer-Chicko
authors.forename: Sandra
authors.initials: S
authors.lastname: Kaplan
authors.forename: Jeffery S
authors.initials: JS
authors.lastname: Sager
authors.forename: Robert
authors.initials: R
authors.lastname: Gallop
authors.forename: Jason
authors.initials: J
authors.lastname: Christie
authors.forename: John
authors.initials: J
authors.lastname: Hansen-Flaschen
authors.forename: Harold
authors.initials: H
authors.lastname: Palevsky
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003430:Cross-Sectional Studies; D005260:Female; D006304:Health Status; D006305:Health Status Indicators; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D010045:Outpatients / Q000706:statistics & numerical data*; D010414:Pennsylvania / Q000453:epidemiology; D011788:Quality of Life; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors