title: Health-related quality of life in a national cohort of patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.
abstract: BACKGROUND
  Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) experience impaired health-related quality of life (HRQL). The objective of this study was to evaluate HRQL in a nation-wide sample.
  
  
  METHODS
  This is a prospective, multicenter, non-interventional study of HRQL including 139 (89%) PAH and 17 (11%) CTEPH patients (women 70.5%; mean age, 52.2) recruited from 21 Spanish hospitals. 55% had idiopathic PAH, 34% other PAH and 11% CTEPH. HRQL was measured using the Short Form 36 Health Survey (SF-36) and EuroQoL-5D (baseline and after 6 months).
  
  
  RESULTS
  HRQL in the patients with PAH or CTEPH was impaired. The physical component of SF-36 and the EuroQol-5D correlated with the functional class (FC). Mean EuroQol-5D visual analogical scale (EQ-5D VAS) scores were 73.5±18.4, 62.9±20.7 and 51.3±16.0 (P<.0001) in patients with FC I, II and III, respectively. Every increase of one FC represented a loss of 4.0 on the PCS SF-36 and a loss of 9.5 on the EQ-5D VAS. Eight patients who died or received a transplant during the study period presented poorer initial HRQL compared with the rest of the population. No significant changes in HRQL were observed in survivors after 6 months of follow-up.
  
  
  CONCLUSIONS
  HRQL is impaired in this population, especially in PAH/CTEPH patients near death. HRQL measurements could help predict the prognosis in PAH and CTPH and provide additional information in these patients.
authors: Hospital Vall d'Hebrón, CIBERES, Barcelona, España. aroman@vhebron.net
authors: Antonio
authors: A
authors: Roman
authors: Joan Albert
authors: JA
authors: Barbera
authors: Maria Jesús
authors: MJ
authors: Castillo
authors: Rocío
authors: R
authors: Muñoz
authors: Pilar
authors: P
authors: Escribano
mesh_terms: D000328:Adult; D000368:Aged; D015331:Cohort Studies; D018450:Disease Progression; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006306:Health Surveys; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality / Q000523:psychology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011655:Pulmonary Embolism / Q000150:complications*; D011788:Quality of Life; D012720:Severity of Illness Index; D013030:Spain / Q000453:epidemiology; D011795:Surveys and Questionnaires