title: Evaluation of disease-specific health-related quality of life in patients with pulmonary arterial hypertension.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) remains a debilitating and life-threatening disease despite improvements in hemodynamics, exercise capacity and survival with recent therapeutic advances. Health-related quality of life (HRQOL) has, therefore, been proposed as an important outcome for evaluating care. Relatively little, however, is known regarding HRQOL or its determinants in PAH. The Minnesota Living with Heart Failure questionnaire was recently adapted and validated for HRQOL measurement in PAH. We applied this pulmonary hypertension-specific version (MLHF-PH) to a larger population of PAH patients.
  
  
  METHODS
  Ninety-three consecutive outpatients with PAH completed the MLHF-PH. Scores were assessed for correlations with demographics, symptoms, hemodynamics and treatments.
  
  
  RESULTS
  Patients with PAH had significantly impaired HRQOL as assessed by the disease-specific MLHF-PH. Each physical and emotional component, as well as total scores on the MLHF-PH indicated severely depressed HRQOL. As compared to other diagnoses, PAH associated with scleroderma had the worst HRQOL. Patients with WHO functional Class II symptoms reported better HRQOL than Class III patients. Fatigue, weakness and abdominal discomfort were each associated with more severely depressed HRQOL, as was current epoprostenol use. With the sole exception of the right atrial pressure, hemodynamic measurements did not correlate with HRQOL scores. Simultaneous evaluation of HRQOL with a non-disease-specific questionnaire (SF-36) revealed a similarly impaired status, although identified fewer associations with patient-specific factors.
  
  
  CONCLUSIONS
  Severely impaired HRQOL is present in this population of patients with PAH evaluated with a disease-specific questionnaire. The availability of a pulmonary hypertension-specific HRQOL questionnaire may enable further targeted investigations of factors that might improve outcomes.
authors.affiliation: Pulmonary, Allergy and Critical Care Division, Department of Medicine, University of Pennsylvania School of Medicine, Penn Presbyterian Medical Center, Suite 120, 51 North 39th Street, Philadelphia, PA 19104, USA. mzlupko@gmail.com
authors.forename: Michael
authors.initials: M
authors.lastname: Zlupko
authors.forename: Michael O
authors.initials: MO
authors.lastname: Harhay
authors.forename: Robert
authors.initials: R
authors.lastname: Gallop
authors.forename: Jennifer
authors.initials: J
authors.lastname: Shin
authors.forename: Chris
authors.initials: C
authors.lastname: Archer-Chicko
authors.forename: Rajesh
authors.initials: R
authors.lastname: Patel
authors.forename: Harold I
authors.initials: HI
authors.lastname: Palevsky
authors.forename: Darren B
authors.initials: DB
authors.lastname: Taichman
mesh_terms: D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000704:Analysis of Variance; D002637:Chest Pain; D011464:Epoprostenol / Q000009:adverse effects / Q000627:therapeutic use; D005221:Fatigue; D005260:Female; D006304:Health Status; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000503:physiopathology / Q000523:psychology*; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D018434:Sickness Impact Profile; D014859:Warfarin / Q000009:adverse effects / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D014859:Warfarin; D011464:Epoprostenol