PMID- 20580264
OWN - NLM
STAT- MEDLINE
DCOM- 20110114
LR  - 20181201
IS  - 1557-3117 (Electronic)
IS  - 1053-2498 (Linking)
VI  - 29
IP  - 10
DP  - 2010 Oct
TI  - Long-term outcomes in pulmonary arterial hypertension in the first-line 
      epoprostenol or first-line bosentan era.
PG  - 1150-8
LID - 10.1016/j.healun.2010.05.011 [doi]
AB  - BACKGROUND: The aim of this study was to describe the long-term outcomes in 
      idiopathic pulmonary arterial hypertension (IPAH) treated with first-line 
      bosentan or intravenous (IV) epoprostenol, and additional therapy as needed. 
      METHODS: In a single-center, retrospective, longitudinal cohort, data on right 
      heart catheterization, 6-minute walk distance (6MWD), disease progression and 
      mortality were collected. Outcomes were assessed in first-line bosentan and 
      first-line epoprostenol patients. To reduce selection bias due to differences 
      between groups, two independent analyses were performed. First, a comparison was 
      made of World Health Organization (WHO) Functional Class (FC) III patients. 
      Second, to control for disease severity, a matched-pairs analysis was performed, 
      with matching according to baseline cardiac output and exercise capacity and 
      irrespective of FC at baseline. RESULTS: Thirty-seven IPAH patients initiated 
      first-line bosentan treatment and 37 first-line IV epoprostenol. Twenty-nine of 
      the bosentan patients and 16 of the IV epoprostenol patients were in WHO FC III; 
      demographic profiles were similar, although hemodynamic measurements and 6MWD 
      suggested more severe disease in the IV epoprostenol group at treatment 
      initiation. At 1 and 3 years, median change in 6MWD for patients initiating 
      bosentan was +54 m (95% confidence interval: -3 to 76) and +71 m (-123 to 116), 
      respectively, and +92 m (17 to 128) and +142 m (-6 to 242) for those on IV 
      epoprostenol. Absence of disease progression of WHO FC III at 1 and 3 years was 
      72% and 45% with bosentan and 75% and 44% with IV epoprostenol, respectively. 
      Survival at 1 and 3 years was 93% and 89% with bosentan and 94% and 75% with IV 
      epoprostenol, respectively. Results were confirmed in matched-pairs analysis of 
      16 bosentan and 16 IV epoprostenol patients with similar disease severity. 
      CONCLUSIONS: First-line epoprostenol treatment may lead to greater improvement in 
      exercise capacity than first-line bosentan. However, these greater exercise 
      improvements did not translate into longer time to disease progression or 
      survival.
CI  - Copyright (c) 2010 International Society for Heart and Lung Transplantation. 
      Published by Elsevier Inc. All rights reserved.
FAU - Jacobs, Wouter
AU  - Jacobs W
AD  - Department of Pulmonology, VU University Medical Center, Amsterdam, The 
      Netherlands.
FAU - Boonstra, Anco
AU  - Boonstra A
FAU - Brand, Monika
AU  - Brand M
FAU - Rosenberg, Daniel M
AU  - Rosenberg DM
FAU - Schaaf, Berthold
AU  - Schaaf B
FAU - Postmus, Pieter E
AU  - Postmus PE
FAU - Vonk Noordegraaf, Anton
AU  - Vonk Noordegraaf A
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20100626
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/*therapeutic use
MH  - Bosentan
MH  - Epoprostenol/*therapeutic use
MH  - Exercise Tolerance
MH  - Humans
MH  - Hypertension, Pulmonary/*drug therapy/mortality/pathology
MH  - Longitudinal Studies
MH  - Male
MH  - Middle Aged
MH  - Retrospective Studies
MH  - Sulfonamides/*therapeutic use
MH  - Survival Analysis
MH  - Treatment Outcome
EDAT- 2010/06/29 06:00
MHDA- 2011/01/15 06:00
CRDT- 2010/06/29 06:00
PHST- 2009/11/30 00:00 [received]
PHST- 2010/04/16 00:00 [revised]
PHST- 2010/05/06 00:00 [accepted]
PHST- 2010/06/29 06:00 [entrez]
PHST- 2010/06/29 06:00 [pubmed]
PHST- 2011/01/15 06:00 [medline]
AID - S1053-2498(10)00292-5 [pii]
AID - 10.1016/j.healun.2010.05.011 [doi]
PST - ppublish
SO  - J Heart Lung Transplant. 2010 Oct;29(10):1150-8. doi: 
      10.1016/j.healun.2010.05.011. Epub 2010 Jun 26.