PT  - JOURNAL ARTICLE
AU  - Pascal Amedro
AU  - Adeline Basquin
AU  - Virginie Gressin
AU  - Pierre Clerson
AU  - Xavier Jais
AU  - Jean-Benoit Thambo
AU  - Damien Bonnet
TI  - Quality of life of patients with pulmonary arterial hypertension associated with congenital heart disease: The multicenter cross-sectional ACHILLE study
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P2384
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P2384.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P2384.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Objectives: To assess health-related quality of life (QoL) in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) and correlations with clinical status.Methods: This cross-sectional study included PAH-CHD patients in 14 centers in France. QoL was self-reported with a generic questionnaire (SF-36) and a PH-specific questionnaire (CAMPHOR). Patients filled out the Hospital Anxiety and Depression Scale (HADS) questionnaire. Main clinical data were collected.Results: 200 patients were included (mean age 43±SD15.7 years, range 15 to 86 years, 70% female), and were classified as Eisenmenger syndromes (73.5%), PAH associated with systemic to pulmonary shunts (13%), PAH associated with small defects (3.5%) and PAH after corrective cardiac surgery (10%). At inclusion, 76.5% patients were receiving PAH-specific treatments. All component scores of both QoL questionnaires showed degradation vs healthy population standard scores. QoL scores were lower in females than in males for all dimensions of both questionnaires (p<0.05). QoL scores were independent of the type of PAH-CHD but significantly depended on NYHA class even after gender adjustment. In a multivariate analysis, variables significantly affecting QoL included NYHA class, six-minute walk distance, HADS, gender, recent stressful event, and more than one hospitalization within the past 12 months.Conclusion: This study showed degradation of QoL in a large cohort of PAH-CHD patients with both generic and specific questionnaires. QoL was correlated with NYHA class but not with the type of PAH-CHD.