Document Detail

Effects of long-term bosentan in children with pulmonary arterial hypertension.
MedLine Citation:
PMID:  16098438     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: This study investigated the long-term outcome of children with pulmonary arterial hypertension (PAH) treated with bosentan therapy, with or without concomitant prostanoid therapy.
BACKGROUND: Bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, improves hemodynamics and exercise capacity in adults with PAH; however, limited data are available on its long-term effects in children.
METHODS: In this retrospective study, 86 children with PAH (idiopathic, associated with congenital heart or connective tissue disease) started bosentan with or without concomitant intravenous epoprostenol or subcutaneous treprostinil therapy. Hemodynamics, World Health Organization (WHO) functional class, and safety data were collected.
RESULTS: At the cutoff date, 68 patients (79%) were still treated with bosentan, 13 (15%) were discontinued, and 5 (6%) had died. Median exposure to bosentan was 14 months. In 90% of the patients (n = 78), WHO functional class improved (46%) or was unchanged (44%) with bosentan treatment. Mean pulmonary artery pressure and pulmonary vascular resistance decreased (64 +/- 3 mm Hg to 57 +/- 3 mm Hg, p = 0.005 and 20 +/- 2 U x m2 to 15 +/- 2 U x m2, p = 0.01, respectively; n = 49). Kaplan-Meier survival estimates at one and two years were 98% and 91%, respectively. The risk for worsening PAH was lower in patients in WHO functional class I/II at bosentan initiation than in patients in WHO class III/IV at bosentan initiation.
CONCLUSIONS: These data suggest that bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, with or without concomitant prostanoid therapy, is safe and efficacious for the treatment of PAH in children.
Erika Berman Rosenzweig; D Dunbar Ivy; Allison Widlitz; Aimee Doran; Lori R Claussen; Delphine Yung; Steven H Abman; Adele Morganti; Ngoc Nguyen; Robyn J Barst
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  46     ISSN:  0735-1097     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  2005 Aug 
Date Detail:
Created Date:  2005-08-15     Completed Date:  2005-09-13     Revised Date:  2014-09-08    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  697-704     Citation Subset:  AIM; IM    
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MeSH Terms
Antihypertensive Agents / adverse effects,  therapeutic use*
Child, Preschool
Drug Therapy, Combination
Hypertension, Pulmonary / drug therapy*,  mortality
Prostaglandins / administration & dosage,  therapeutic use
Receptor, Endothelin A / antagonists & inhibitors*
Receptor, Endothelin B / antagonists & inhibitors*
Retrospective Studies
Risk Assessment
Risk Factors
Sulfonamides / adverse effects,  therapeutic use*
Survival Analysis
Treatment Outcome*
Grant Support
M01 RR000069/RR/NCRR NIH HHS; M01 RR000069-457038/RR/NCRR NIH HHS; M01 RR00069/RR/NCRR NIH HHS
Reg. No./Substance:
0/Antihypertensive Agents; 0/Prostaglandins; 0/Receptor, Endothelin A; 0/Receptor, Endothelin B; 0/Sulfonamides; Q326023R30/bosentan
Comment In:
J Am Coll Cardiol. 2005 Aug 16;46(4):705-6   [PMID:  16098439 ]
J Am Coll Cardiol. 2006 May 2;47(9):1914-5; author reply 1915   [PMID:  16682327 ]

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