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.

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.

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.

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.