Document Detail


Effects of long-term bosentan in children with pulmonary arterial hypertension.
MedLine Citation:
PMID:  16098438     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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
Related Documents :
18753548 - 474562 - beta blockers increase perioperative risk in acute anemia surgery.
18671148 - Survival with tiotropium compared to long-acting beta-2-agonists in chronic obstructive...
15190528 - A phase ii, double-blind, randomized, placebo-controlled, dose comparative study of the...
15704058 - Clinical effects of endothelin receptor antagonism with bosentan in patients with sever...
20113698 - Therapeutic value of sesame oil in the treatment of adhesive small bowel obstruction.
25470818 - Evaluation of safety and efficacy of intravenous zanamivir in the treatment of hospital...
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    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adolescent
Antihypertensive Agents / adverse effects,  therapeutic use*
Child
Child, Preschool
Drug Therapy, Combination
Female
Humans
Hypertension, Pulmonary / drug therapy*,  mortality
Male
Prostaglandins / administration & dosage,  therapeutic use
Receptor, Endothelin A / antagonists & inhibitors*
Receptor, Endothelin B / antagonists & inhibitors*
Retrospective Studies
Risk Assessment
Risk Factors
Safety
Sulfonamides / adverse effects,  therapeutic use*
Survival Analysis
Time
Treatment Outcome*
Grant Support
ID/Acronym/Agency:
M01 RR000069/RR/NCRR NIH HHS; M01 RR000069-457038/RR/NCRR NIH HHS; M01 RR00069/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/Antihypertensive Agents; 0/Prostaglandins; 0/Receptor, Endothelin A; 0/Receptor, Endothelin B; 0/Sulfonamides; Q326023R30/bosentan
Comments/Corrections
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 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Prevalence and clinical significance of left atrial remodeling in competitive athletes.
Next Document:  Plasma tissue factor plus activated peripheral mononuclear cells activate factors VII and X in cardi...