Pulmonary hypertension due to lung disease – Results from COMPERA

Introduction: COMPERA is a European registry that prospectively enrols patients with various forms of pulmonary hypertension (PH) receiving targeted therapy. Aims and objectives: We compared patient characteristics, haemodynamics, treatment patterns and outcomes of patients with severe PH due to lung disease and patients with idiopathic pulmonary arterial hypertension (IPAH). Methods: Data were analysed from patients enrolled within 6 months of newly diagnosed PH into COMPERA until February 2015. Results: 1,472 patients were eligible for this analysis: IPAH, n=1,047; PH due to chronic obstructive lung disease (PH-COPD), n=260; and PH due to interstitial lung disease (PH-ILD), n=165. As shown in the table, all patient groups were characterized by severe PH and severe functional impairment. All patients received PH targeted therapies with pulmonary vasodilators; patients with PH due to lung disease mostly (90%) with PDE5i monotherapy. Survival rates at 1, 2 and 3 years were 91.7%, 81.5% and 70.7% in IPAH; 86.6%, 76.5% and 58.8% in PH-COPD; and 73.3%, 51.2% and 34.1% in PH-ILD (p Conclusions: In the COMPERA registry patients with PH due to lung disease presented with severe haemodynamic and functional impairment. Most patients received PDE5i-monotherapy. Despite targeted therapy patients with PH-COPD or PH-ILD had a significant worse survival than patients with IPAH. The effects of PH therapy on symptoms and outcomes in these patients require further studies.