title: Quality of life as a prognostic marker in pulmonary arterial hypertension.
abstract: BACKGROUND
  Improvement in quality of life together with better survival are the ultimate goals in the treatment of pulmonary arterial hypertension (PAH) patients. The objective of this study was to evaluate the health-related quality of life (HRQL) of pulmonary arterial hypertension (PAH) patients with the SF-36 generic questionnaire and to identify the prognostic implication of this assessment.
  
  
  METHODS
  Fifty-four consecutive newly diagnosed PAH patients (WHO classification group I) in a single PAH reference center were included. Patients were evaluated at baseline for clinical and hemodynamic parameters, and they subsequently received first-line therapy with either an endothelin receptor antagonist or a phosphodiesterase-5 inhibitor. After 16 weeks of specific PAH therapy, all patients were re-evaluated using a 6MWT and a SF 36 questionnaire, and then they were followed up for at least 36 months.
  
  
  RESULTS
  After treatment, the patients demonstrated an improved 6MWT (414 ± 124 m vs. 440 ± 113 m, p = 0.001). Specific PAH therapy also improved the HRQL scores.Patients with a baseline Physical Component Score (PCS) higher than 32 had a better survival rate than those who had a score under 32 (p = 0.04). Similarly, patients with a PCS of at least a 38 after the 16 week therapy period had a better survival rate when compared with those who did not achieve this value (p = 0.016). Unlike the absolute PCS values, the post-treatment PCS variability was unable to predict better survival rates (p = 0.58).
  
  
  CONCLUSIONS
  Our findings suggest that HRQL is associated with prognosis in PAH. Furthermore, achieving pre-determined PCS scores might represent a specific goal to be reached in treatment-to-target strategies.
authors.forename: Caio J C S
authors.initials: CJ
authors.lastname: Fernandes
authors.forename: Barbara C S
authors.initials: BC
authors.lastname: Martins
authors.forename: Carlos V P
authors.initials: CV
authors.lastname: Jardim
authors.forename: Rozana M
authors.initials: RM
authors.lastname: Ciconelli
authors.forename: Luciana K
authors.initials: LK
authors.lastname: Morinaga
authors.forename: Ana Paula
authors.initials: AP
authors.lastname: Breda
authors.forename: Susana
authors.initials: S
authors.lastname: Hoette
authors.affiliation: Pulmonary Department, Heart Institute - University of Sao Paulo Medical School, Medical School, Av, Dr, Eneas de Carvalho Aguiar, 44, Sao Paulo, 05403-000, Brazil. souza.rogerio@me.com.
authors.forename: Rogério
authors.initials: R
authors.lastname: Souza
mesh_terms: D000328:Adult; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006305:Health Status Indicators; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D011788:Quality of Life; D011795:Surveys and Questionnaires; D015996:Survival Rate
chemical_list: D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors