RT期刊文章SR电子T1前期三倍combination therapy in pulmonary arterial hypertension: a pilot study JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 1691 OP 1697 DO 10.1183/09031936.00116313 VO 43 IS 6 A1 Sitbon, Olivier A1 Jaïs, Xavier A1 Savale, Laurent A1 Cottin, Vincent A1 Bergot, Emmanuel A1 Macari, Elise Artaud A1 Bouvaist, Hélène A1 Dauphin, Claire A1 Picard, François A1 Bulifon, Sophie A1 Montani, David A1 Humbert, Marc A1 Simonneau, Gérald YR 2014 UL //www.qdcxjkg.com/content/43/6/1691.abstract AB Patients with severe pulmonary arterial hypertension (PAH) in New York Heart Association (NYHA) functional class (FC) III/IV have a poor prognosis, despite survival benefits being demonstrated with intravenous epoprostenol. In this pilot study, the efficacy and safety of a triple combination therapy regimen in patients with severe PAH was investigated. Data from newly diagnosed NYHA FC III/IV PAH patients (n=19) initiated on upfront triple combination therapy (intravenous epoprostenol, bosentan and sildenafil) were collected retrospectively from a prospective registry. Significant improvements in 6-min walk distance and haemodynamics were observed after 4 months’ triple combination therapy in 18 patients (p<0.01); 17 patients had improved to NYHA FC I or II. One patient was not included in the month 4 assessment (due to an emergency lung transplant in month 3). At the final evaluation (mean±sd 32±19 months), all 18 patients had sustained clinical and haemodynamic improvement. Overall survival estimates for the triple combination cohort were 100% at 1, 2 and 3 years. Expected survival calculated from the French equation was 75% (95% CI 68–82%), 60% (95% CI 50–70%) and 49% (95% CI 38–60%) at 1, 2 and 3 years, respectively. This pilot study provides preliminary evidence of the long-term benefits of upfront triple combination therapy in patients with severe PAH. Upfront triple combination therapy shows promising long-term efficacy in severe pulmonary arterial hypertension http://ow.ly/tKgHX