TY - JOUR T1 - Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study JF - European Respiratory Journal JO - Eur Respir J SP - 1691 LP - 1697 DO - 10.1183/09031936.00116313 VL - 43 IS - 6 AU - Olivier Sitbon AU - Xavier Jaïs AU - Laurent Savale AU - Vincent Cottin AU - Emmanuel Bergot AU - Elise Artaud Macari AU - Hélène Bouvaist AU - Claire Dauphin AU - François Picard AU - Sophie Bulifon AU - David Montani AU - Marc Humbert AU - Gérald Simonneau Y1 - 2014/06/01 UR - http://erj.ersjournals.com/content/43/6/1691.abstract N2 - 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 ER -