TY - JOUR T1 - Current epoprostenol use in patients with severe pulmonary hypertension (PH): Data from the French PH registry JF - European Respiratory Journal JO - Eur Respir J VL - 38 IS - Suppl 55 SP - p2382 AU - Emmanuel Bergot AU - Olivier Sitbon AU - Vincent Cottin AU - Azzedine Yaici AU - Grégoire Prévot AU - Laurence Rottat AU - Virginie Gressin AU - Marc Humbert AU - Gérald Simonneau Y1 - 2011/09/01 UR - http://erj.ersjournals.com/content/38/Suppl_55/p2382.abstract N2 - The management of pulmonary arterial hypertension (PAH) has evolved since the early 2000s with the introduction of oral therapies, and consequently data on the current use of epoprostenol (epo) are scarce.Methods: Patients (pts) with a newly diagnosed PH treated with epo were analysed from the prospective French PH Registry launched in Nov. 2006. The cut-off date was 1 Sept. 2010.Results: 177 adult pts were identified for analysis. 127 (72%) had PAH (group 1) and among them, 81 (64%) had idiopathic, heritable or anorexigen-associated PAH (IHAr-PAH), 17 (13%) connective tissue disease and 17 (13%) portopulmonary hypertension. 6% of patients received epo for pulmonary veno-occlusive disease, 8% for PH with lung diseases, 12% for chronic thromboembolic PH and 2% for miscellaneous PH. At the time of epo initiation, mean (±SD) age was 51±17 yrs. 7%, 43% and 50% of pts were in NYHA class II, III, IV, respectively, and 47% were naive to PAH specific therapy. The 6-min walk distance was 309±131 m. Haemodynamic measures confirmed severe impairment with mean PAP 56±13 mmHg, right atrial pressure 10±6 mmHg, cardiac index 2.0±0.6 L/min/m2 and pulmonary vascular resistance 1194±571 dyn s cm-5. In the overall PH population, survival estimates following epo commencement were 77%, 63% and 54% at 1, 2 and 3 years, respectively, and 82%, 72% and 69% in the subgroup of pts with IHAr-PAH.Conclusion: Epo is still used as first-line therapy in severe forms of PAH. It is notably prescribed in non-group 1 PH in 28% of cases. In incident pts with IHAr-PAH, 1- and 2-year survival is similar to that of historical cohorts, despite the prior use of an oral treatment in 48% percent of them. ER -