PMID- 22138355 OWN - NLM STAT- MEDLINE DCOM- 20120523 LR - 20211223 IS - 1557-3117 (Electronic) IS - 1053-2498 (Linking) VI - 31 IP - 2 DP - 2012 Feb TI - Usefulness of first-line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension: an observational study. PG - 150-8 LID - 10.1016/j.healun.2011.11.002 [doi] AB - BACKGROUND: Recent guidelines have proposed first-line combination therapy as a potential strategy for the treatment of functional class IV pulmonary arterial hypertension (PAH). METHODS: We analyzed efficacy and safety of upfront epoprostenol and bosentan combination therapy in consecutive patients with idiopathic, heritable, or anorexigen-associated PAH and compared outcomes with matched controls treated by epoprostenol monotherapy. RESULTS: Data for 16 functional class III patients and 7 functional class IV patients were analyzed. Baseline 6-minute walk distance (6MWD) was 287 +/- 133 meters, mean pulmonary artery pressure was 65 +/- 12 mm Hg, cardiac index was 1.8 +/- 0.3 L/min/m(2), and pulmonary vascular resistance (PVR) was 1493 +/- 398 dynes/sec/cm(5). After 4 months, 6MWD and PVR significantly improved to 421 +/- 100 meters and 784 +/- 364 dynes/sec/cm(5), respectively. These improvements were maintained long-term (30 +/- 19 months). At 1, 2, 3, and 4 years, overall survival estimates were 100%, 94%, 94%, and 74%, and transplant-free survival estimates were 96%, 85%, 77%, and 60%, respectively. Compared with matched controls started on epoprostenol monotherapy, there was a trend to an improvement in overall survival (p = 0.07). CONCLUSIONS: Initial combination therapy with epoprostenol and bosentan in patients with severe PAH is associated with improvements in important outcomes such as functional class, exercise capacity, and hemodynamics. This combination strategy might also favorably affect overall and transplant-free survival. CI - Copyright (c) 2012 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved. FAU - Kemp, Kristina AU - Kemp K AD - Universite Paris-Sud 11, Faculte de Medecine, Le Kremlin-Bicetre, Clamart, France. FAU - Savale, Laurent AU - Savale L FAU - O'Callaghan, Dermot S AU - O'Callaghan DS FAU - Jais, Xavier AU - Jais X FAU - Montani, David AU - Montani D FAU - Humbert, Marc AU - Humbert M FAU - Simonneau, Gerald AU - Simonneau G FAU - Sitbon, Olivier AU - Sitbon O LA - eng PT - Journal Article DEP - 20111203 PL - United States TA - J Heart Lung Transplant JT - The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation JID - 9102703 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - DCR9Z582X0 (Epoprostenol) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Antihypertensive Agents/administration & dosage/adverse effects/*therapeutic use MH - Bosentan MH - Case-Control Studies MH - Drug Therapy, Combination MH - Epoprostenol/administration & dosage/adverse effects/*therapeutic use MH - Familial Primary Pulmonary Hypertension MH - Female MH - Humans MH - Hypertension, Pulmonary/*drug therapy/physiopathology MH - Injections, Intravenous MH - Male MH - Middle Aged MH - Retrospective Studies MH - Severity of Illness Index MH - Sulfonamides/administration & dosage/adverse effects/*therapeutic use MH - Treatment Outcome EDAT- 2011/12/06 06:00 MHDA- 2012/05/24 06:00 CRDT- 2011/12/06 06:00 PHST- 2011/01/17 00:00 [received] PHST- 2011/09/12 00:00 [revised] PHST- 2011/11/07 00:00 [accepted] PHST- 2011/12/06 06:00 [entrez] PHST- 2011/12/06 06:00 [pubmed] PHST- 2012/05/24 06:00 [medline] AID - S1053-2498(11)01203-4 [pii] AID - 10.1016/j.healun.2011.11.002 [doi] PST - ppublish SO - J Heart Lung Transplant. 2012 Feb;31(2):150-8. doi: 10.1016/j.healun.2011.11.002. Epub 2011 Dec 3.