0238: Quality of life of patients with pulmonary arterial hypertension associated with congenital heart disease: the multicenter cross-sectional Achille study

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 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.