Abstract | BACKGROUND: METHODS: WHO functional class, resting oxygen saturation, 6-minute walk test (6 MWT) and hemodynamics were assessed at baseline and after 12 months 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 12 months 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 ± 74 m, p=0.0007; controls: 389 ± 80 vs 343 ± 86 m, p=0.00003), and hemodynamics (pulmonary flow, Down: 4.0 ± 1.6 vs 3.5 ± 1.4 l/m/m(2), p=0.006; controls: 3.5 ± 1.4 vs 2.8 ± 1.0 l/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 ± 13 WUm(2), p=0.007; controls: 2 0 ± 10 vs 26 ± 15 WUm(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 12 months of treatment. Clinical status, exercise tolerance, and pulmonary hemodynamics improved, regardless of the presence of Down's syndrome.
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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 |
Journal | International journal of cardiology
(Int J Cardiol)
Vol. 164
Issue 3
Pg. 323-6
(Apr 15 2013)
ISSN: 1874-1754 [Electronic] Netherlands |
PMID | 21802156
(Publication Type: Clinical Trial, Comparative Study, Journal Article)
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Copyright | Copyright © 2011 Elsevier Ireland Ltd. All rights reserved. |
Chemical References |
- Antihypertensive Agents
- Sulfonamides
- Bosentan
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Topics |
- Administration, Oral
- Adult
- Antihypertensive Agents
(administration & dosage)
- Bosentan
- Down Syndrome
(complications)
- Exercise Tolerance
(drug effects)
- Familial Primary Pulmonary Hypertension
- Female
- Follow-Up Studies
- Heart Defects, Congenital
(complications)
- Hemodynamics
(drug effects)
- Humans
- Hypertension, Pulmonary
(drug therapy, etiology)
- Male
- Middle Aged
- Prospective Studies
- Sulfonamides
(administration & dosage)
- Treatment Outcome
- Young Adult
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