title: Health-related quality of life of patients with pulmonary arterial hypertension associated with CHD: the multicentre cross-sectional ACHILLE study.
abstract: BACKGROUND
  The aim of this study was to assess health-related quality of life in patients with pulmonary arterial hypertension associated with CHD and correlations with clinical status.
  
  
  METHODS
  This prospective cross-sectional observational study included CHD patients with pulmonary arterial hypertension in 14 tertiary-care centres in France. We used two health-related quality of life questionnaires - SF-36 and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) - and one anxiety/depression Hospital Anxiety and Depression Scale (HADS) questionnaire.
  
  
  RESULTS
  Clinical data were collected for the 208 included patients (mean age: 42.6 years, range from 15.1 to 85.8 years, 69.7% female). Most patients were in NYHA functional class II (48.1%) and III (37.5%). Patients' phenotype was classified as Eisenmenger syndrome (70.7%), pulmonary arterial hypertension associated with systemic-to-pulmonary shunts (12.0%), with small defects (3.4%), or after corrective cardiac surgery (13.9%). In total, 76.4% of the patients were receiving pulmonary arterial hypertension-specific treatments. SF-36 scores showed impairment compared with normalised data. Health-related quality of life scores were significantly lower in females than in males for most dimensions of both questionnaires and were independent of the patients' phenotype, even after gender adjustment - except for CAMPHOR functioning - but significantly depended on NYHA functional class. The Hospital Anxiety and Depression Scale (HADS) scores suggested anxiety and depression associated with increasing NYHA functional class but independent of patients' phenotype. NYHA functional class, 6-minute walk distance, HADS, gender, and recent stressful event significantly affected quality of life in the multivariate analysis.
  
  
  CONCLUSIONS
  This study showed impairment of quality of life in a large cohort of patients with pulmonary arterial hypertension associated with CHD with both generic and specific questionnaires. NYHA functional class and HADS scores were predictive of most quality of life scores.
authors.affiliation: 1Pediatric and Congenital Cardiology,Arnaud de Villeneuve University Hospital,PhyMedexp,INSERM U1046,CNRS UMR 9214,University of Montpellier,Montpellier,France.
authors.forename: Pascal
authors.identifier: http://orcid.org/0000-0003-3649-0294
authors.initials: P
authors.lastname: Amedro
authors.affiliation: 3Pediatric and Congenital Cardiology,University Hospital,Rennes,France.
authors.forename: Adeline
authors.initials: A
authors.lastname: Basquin
authors.affiliation: 4Actelion Pharmaceuticals France,Paris,France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Gressin
authors.affiliation: 5Orgametrie Biostatistics,Roubaix,France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Clerson
authors.affiliation: 6Bicêtre University Hospital,Le Kremlin-Bicêtre,France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Jais
authors.affiliation: 7Pediatric and Congenital Cardiology,Haut-Leveque University Hospital,Bordeaux,France.
authors.forename: Jean-Benoit
authors.initials: JB
authors.lastname: Thambo
authors.affiliation: 8Pediatric and Congenital Cardiology,Guillaume and René Laennec University Hospital,Nantes,France.
authors.forename: Patrice
authors.initials: P
authors.lastname: Guerin
authors.affiliation: 9Adult Congenital Heart Disease Unit,M3C-George Pompidou European Hospital,AP-HP,Paris Descartes University,France.
authors.forename: Sarah
authors.initials: S
authors.lastname: Cohen
authors.affiliation: 11Pediatric and Congenital Cardiology,M3C-Necker University Hospital,AP-HP,Paris Descartes University,Paris, France,France.
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001007:Anxiety / Q000453:epidemiology*; D003430:Cross-Sectional Studies; D003863:Depression / Q000453:epidemiology*; D004541:Eisenmenger Complex / Q000150:complications* / Q000453:epidemiology*; D065627:Familial Primary Pulmonary Hypertension / Q000453:epidemiology*; D005260:Female; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011446:Prospective Studies; D011569:Psychiatric Status Rating Scales; D011788:Quality of Life; D012044:Regression Analysis; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: CAMPHOR; CHD; Eisenmenger syndrome; Health-related quality of life; pulmonary arterial hypertension