| Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome. | |
| | |
MedLine Citation:
|
PMID: 21802156 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
|
BACKGROUND: Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down's syndrome, the effect of bosentan is largely unknown. Aim of the study was to evaluate the long-term effects of bosentan in adult patients with CHD-related PAH with and without Down's syndrome. METHODS: WHO functional class, resting oxygen saturation, 6-minute walk test (6MWT) and hemodynamics were assessed at baseline and after 12months of bosentan therapy in patients with CHD-related PAH with and without Down's syndrome. RESULTS: Seventy-four consecutive patients were enrolled: 18 with and 56 without Down's syndrome. After 12months of bosentan therapy, both with and without Down's syndrome patients showed an improvement in WHO functional class (Down: 2.5±0.5 vs 2.9±0.6, p=0.005; controls: 2.5±0.5 vs 2.9±0.5, p=0.000002), 6-minute walk distance (Down: 288±71 vs 239±74m, p=0.0007; controls: 389±80 vs 343±86m, p=0.00003), and hemodynamics (pulmonary flow, Down: 4.0±1.6 vs 3.5±1.4l/m/m(2), p=0.006; controls: 3.5±1.4 vs 2.8±1.0l/m/m(2), p=0.0005; pulmonary to systemic flow ratio, Down: 1.4±0.7 vs 1.0±0.4, p=0.003; controls: 1.1±0.7 vs 0.9±0.3, p=0.012; pulmonary vascular resistance index, Down: 15±9 vs 20±13WUm(2), p=0.007; controls: 20±10 vs 26±15WUm(2), p=0.002). No differences in the efficacy of therapy were observed between the two groups. CONCLUSIONS: Bosentan was safe and well tolerated in adult patients with CHD-related PAH with and without Down's syndrome during 12months of treatment. Clinical status, exercise tolerance, and pulmonary hemodynamics improved, regardless of the presence of Down's syndrome. |
| | |
Authors:
|
Michele D'Alto; Emanuele Romeo; Paola Argiento; Antonello D'Andrea; Berardo Sarubbi; Anna Correra; Giancarlo Scognamiglio; Silvia Papa; Eduardo Bossone; Raffaele Calabrò; Carmine D Vizza; Maria G Russo |
Related Documents
:
|
7496176 - Generalized lysosomal storage in yunis varón syndrome. 16767556 - Reactive hemophagocytic syndrome in a case of systemic lupus erythematosus that was dia... 11097286 - Susac syndrome: microangiopathy of the retina, cochlea and brain. 6335386 - Brucellosis and rheumatic syndromes in saudi arabia. 20980806 - New insights into 5q- syndrome as a ribosomopathy. 10533026 - Filippi syndrome: report of three additional cases. |
Publication Detail:
|
Type: JOURNAL ARTICLE Date: 2011-7-27 |
Journal Detail:
|
Title: International journal of cardiology Volume: - ISSN: 1874-1754 ISO Abbreviation: - Publication Date: 2011 Jul |
Date Detail:
|
Created Date: 2011-8-1 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 8200291 Medline TA: Int J Cardiol Country: - |
Other Details:
|
Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
|
Copyright © 2011 Elsevier Ireland Ltd. All rights reserved. |
Affiliation:
|
Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Deleterious sucking habits and atypical swallowing in children with otitis media with effusion.
Next Document: Smoking persistence across countries: A panel data analysis.