PMID- 24627535 OWN - NLM STAT- MEDLINE DCOM- 20150115 LR - 20181202 IS - 1399-3003 (Electronic) IS - 0903-1936 (Linking) VI - 43 IP - 6 DP - 2014 Jun TI - Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. PG - 1691-7 LID - 10.1183/09031936.00116313 [doi] 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. CI - (c)ERS 2014. FAU - Sitbon, Olivier AU - Sitbon O AD - Univ. Paris-Sud, Faculte de Medecine, Le Kremlin-Bicetre APHP, Centre de Reference de l'Hypertension Pulmonaire Severe, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hopital de Bicetre, Le Kremlin-Bicetre INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson olivier.sitbon@bct.aphp.fr. FAU - Jais, Xavier AU - Jais X AD - Univ. Paris-Sud, Faculte de Medecine, Le Kremlin-Bicetre APHP, Centre de Reference de l'Hypertension Pulmonaire Severe, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hopital de Bicetre, Le Kremlin-Bicetre INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson. FAU - Savale, Laurent AU - Savale L AD - Univ. Paris-Sud, Faculte de Medecine, Le Kremlin-Bicetre APHP, Centre de Reference de l'Hypertension Pulmonaire Severe, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hopital de Bicetre, Le Kremlin-Bicetre INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson. FAU - Cottin, Vincent AU - Cottin V AD - Univ. Lyon-1, Hospices Civils de Lyon, Centre de Reference des maladies pulmonaires rares, Centre de Competences de l'Hypertension Pulmonaire, Hopital Louis Pradel, Lyon. FAU - Bergot, Emmanuel AU - Bergot E AD - Service de Pneumologie et d'oncologie thoracique, Hopital Universitaire de Caen, Caen. FAU - Macari, Elise Artaud AU - Macari EA AD - Univ. Paris-Sud, Faculte de Medecine, Le Kremlin-Bicetre APHP, Centre de Reference de l'Hypertension Pulmonaire Severe, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hopital de Bicetre, Le Kremlin-Bicetre INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson. FAU - Bouvaist, Helene AU - Bouvaist H AD - Service de Cardiologie, CHU, Grenoble. FAU - Dauphin, Claire AU - Dauphin C AD - Service de Cardiologie, CHU Gabriel-Montpied, Clermont-Ferrand. FAU - Picard, Francois AU - Picard F AD - Service de Cardiologie, Hopital Haut-Leveque, Pessac, France. FAU - Bulifon, Sophie AU - Bulifon S AD - Univ. Paris-Sud, Faculte de Medecine, Le Kremlin-Bicetre APHP, Centre de Reference de l'Hypertension Pulmonaire Severe, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hopital de Bicetre, Le Kremlin-Bicetre INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson. FAU - Montani, David AU - Montani D AD - Univ. Paris-Sud, Faculte de Medecine, Le Kremlin-Bicetre APHP, Centre de Reference de l'Hypertension Pulmonaire Severe, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hopital de Bicetre, Le Kremlin-Bicetre INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson. FAU - Humbert, Marc AU - Humbert M AD - Univ. Paris-Sud, Faculte de Medecine, Le Kremlin-Bicetre APHP, Centre de Reference de l'Hypertension Pulmonaire Severe, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hopital de Bicetre, Le Kremlin-Bicetre INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson. FAU - Simonneau, Gerald AU - Simonneau G AD - Univ. Paris-Sud, Faculte de Medecine, Le Kremlin-Bicetre APHP, Centre de Reference de l'Hypertension Pulmonaire Severe, Service de Pneumologie et Soins Intensifs, DHU Thorax Innovation, Hopital de Bicetre, Le Kremlin-Bicetre INSERM U999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140313 PL - England TA - Eur Respir J JT - The European respiratory journal JID - 8803460 RN - 0 (Antihypertensive Agents) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfonamides) RN - 0 (Sulfones) RN - BW9B0ZE037 (Sildenafil Citrate) RN - DCR9Z582X0 (Epoprostenol) RN - Q326023R30 (Bosentan) SB - IM CIN - Eur Respir J. 2014 Jun;43(6):1556-9. PMID: 24881056 MH - Adolescent MH - Adult MH - Aged MH - Antihypertensive Agents/*administration & dosage MH - Bosentan MH - Drug Therapy, Combination/methods MH - Epoprostenol/administration & dosage MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/*drug therapy MH - Male MH - Middle Aged MH - Pilot Projects MH - Piperazines/administration & dosage MH - Prognosis MH - Purines/administration & dosage MH - Registries MH - Retrospective Studies MH - Sildenafil Citrate MH - Sulfonamides/administration & dosage MH - Sulfones/administration & dosage MH - Treatment Outcome MH - Young Adult EDAT- 2014/03/15 06:00 MHDA- 2015/01/16 06:00 CRDT- 2014/03/15 06:00 PHST- 2014/03/15 06:00 [entrez] PHST- 2014/03/15 06:00 [pubmed] PHST- 2015/01/16 06:00 [medline] AID - 09031936.00116313 [pii] AID - 10.1183/09031936.00116313 [doi] PST - ppublish SO - Eur Respir J. 2014 Jun;43(6):1691-7. doi: 10.1183/09031936.00116313. Epub 2014 Mar 13.