title: Effect of pulmonary arterial hypertension-specific therapies on health-related quality of life: a systematic review.
abstract: BACKGROUND
  Health-related quality of life (HRQoL) is severely impaired in pulmonary arterial hypertension (PAH). We aimed to assess the effect of PAH-specific therapies on HRQoL.
  
  
  METHODS
  A literature search was performed in MEDLINE and Embase databases (January 1990 to September 2013) to retrieve prospective placebo-controlled randomized trials of at least 6 weeks duration reporting the effect of PAH-specific therapies on HRQoL in adult patients with PAH. The articles were independently reviewed, and the validity of the trials was assessed using the Cochrane's Risk of Bias Tool.
  
  
  RESULTS
  The literature search identified 1,172 titles. Seventeen articles reporting on 14 trials were retrieved, all of which were associated with a low risk of bias. The median study duration of the different trials was 12 weeks. Most patients had idiopathic PAH or PAH associated with connective tissue disease. A variety of HRQoL questionnaires were used in these trials, and most were generic. HRQoL results were most commonly minimally detailed, and some pivotal trials did not even assess HRQoL. Nevertheless, these trials consistently demonstrated statistically significant improvements in HRQoL with PAH-specific therapies, especially for the physical domains. In most cases, however, these improvements were smaller than the minimal important difference in HRQoL previously reported in PAH.
  
  
  CONCLUSIONS
  This review shows that PAH-specific therapies improve HRQoL in PAH. However, it remains difficult to draw any firm conclusion about the clinical significance of these improvements. Further work is mandatory to validate PAH-specific questionnaires that are responsive to clinical changes as well as to establish their interpretability.
authors.affiliation: Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Quebec, QC, Canada; Service de Pneumologie, Centre Hospitalier Universitaire de Besançon, Besançon, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Rival
authors.affiliation: Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Quebec, QC, Canada.
authors.forename: Yves
authors.initials: Y
authors.lastname: Lacasse
authors.affiliation: Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Quebec, QC, Canada.
authors.forename: Sylvie
authors.initials: S
authors.lastname: Martin
authors.affiliation: Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Quebec, QC, Canada.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Bonnet
authors.affiliation: Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Quebec, QC, Canada. Electronic address: Steve.provencher@criucpq.ulaval.ca.
authors.forename: Steeve
authors.initials: S
authors.lastname: Provencher
mesh_terms: D065627:Familial Primary Pulmonary Hypertension / Q000188:drug therapy* / Q000523:psychology*; D006801:Humans; D011788:Quality of Life / Q000523:psychology*; D016032:Randomized Controlled Trials as Topic; D015203:Reproducibility of Results; D011795:Surveys and Questionnaires; D016896:Treatment Outcome