Abstract | BACKGROUND: METHODS: In this open-label study, 24 Down patients (>18 years) with Eisenmenger syndrome (17 males) were treated with bosentan. Their mean age was 38 years (range 19-55 years). All Down patients were evaluated at baseline and during follow-up with laboratory tests, six-minute walk test (6-MWT), Doppler echocardiography, and quality of life questionnaires. RESULTS: The median follow-up of Down patients treated with bosentan was 11.5 months (range 3-23 months). Induction of oral bosentan therapy was well tolerated among all 24 Down patients. Bosentan treatment was generally well tolerated. No serious adverse drug reactions were noted. Median 6-MWT increased from 296 m (range 40-424 m) to 325 m (range 84-459 m, p<0.05) after 12 weeks. After 26 and 52 weeks of treatment with bosentan, median 6-MWT distance was 276 m (range 140-462 m, n=15, p=0.6) and 287 m (range 131-409 m, n=7, p=0.3), respectively. Quality of life questionnaire scores remained stable during treatment. CONCLUSION:
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Authors | Mariëlle G J Duffels, Jeroen C Vis, Rosa L E van Loon, Rolf M F Berger, Elke S Hoendermis, Arie P J van Dijk, Berto J Bouma, Barbara J M Mulder |
Journal | International journal of cardiology
(Int J Cardiol)
Vol. 134
Issue 3
Pg. 378-83
(May 29 2009)
ISSN: 1874-1754 [Electronic] Netherlands |
PMID | 18579234
(Publication Type: Comparative Study, Journal Article)
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Chemical References |
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Topics |
- Adult
- Bosentan
- Down Syndrome
(complications, drug therapy, physiopathology)
- Eisenmenger Complex
(complications, drug therapy, physiopathology)
- Female
- Follow-Up Studies
- Humans
- Male
- Middle Aged
- Sulfonamides
(therapeutic use)
- Treatment Outcome
- Young Adult
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