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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
2009-5-1
pubmed:abstractText
Pulmonary arterial hypertension associated with congenital heart disease caused by systemic-to-pulmonary shunting was associated with a high risk of morbidity and mortality. In this retrospective study, the longer term treatment effect of bosentan on exercise capacity and quality of life (QoL) were evaluated in 58 adult patients (>18 years) with pulmonary arterial hypertension associated with congenital heart disease, including patients with Down's syndrome. All patients were evaluated at baseline and during follow-up using laboratory tests, 6-minute walk test, QoL questionnaires, and Doppler echocardiography. Treatment efficacy was analyzed separately for patients without (n = 30) and with Down's syndrome (n = 28). Median follow-up of all patients treated with bosentan was 22 months (range 3 to 36). In patients without Down's syndrome, mean 6-minute walk distance increased from 427 +/- 97 to 461 +/- 104 m (p <0.01) after 6 months of treatment, followed by a gradual return to baseline and disease stabilization. QoL improved significantly during treatment and was maintained during 18 months of follow-up (p <0.05). In patients with Down's syndrome, 6-minute walk distance and QoL were stable during treatment. In conclusion, findings suggested that in patients without Down's syndrome, longer term bosentan treatment resulted in a persistent improvement in QoL and stabilization of exercise capacity.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
1879-1913
pubmed:author
pubmed:issnType
Electronic
pubmed:day
1
pubmed:volume
103
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1309-15
pubmed:meshHeading
pubmed-meshheading:19406277-Adult, pubmed-meshheading:19406277-Age Factors, pubmed-meshheading:19406277-Aged, pubmed-meshheading:19406277-Antihypertensive Agents, pubmed-meshheading:19406277-Down Syndrome, pubmed-meshheading:19406277-Exercise Test, pubmed-meshheading:19406277-Exercise Tolerance, pubmed-meshheading:19406277-Female, pubmed-meshheading:19406277-Follow-Up Studies, pubmed-meshheading:19406277-Heart Defects, Congenital, pubmed-meshheading:19406277-Humans, pubmed-meshheading:19406277-Hypertension, Pulmonary, pubmed-meshheading:19406277-Magnetic Resonance Imaging, pubmed-meshheading:19406277-Male, pubmed-meshheading:19406277-Middle Aged, pubmed-meshheading:19406277-Probability, pubmed-meshheading:19406277-Quality of Life, pubmed-meshheading:19406277-Retrospective Studies, pubmed-meshheading:19406277-Risk Assessment, pubmed-meshheading:19406277-Severity of Illness Index, pubmed-meshheading:19406277-Sex Factors, pubmed-meshheading:19406277-Sulfonamides, pubmed-meshheading:19406277-Treatment Outcome, pubmed-meshheading:19406277-Young Adult
pubmed:year
2009
pubmed:articleTitle
Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down's syndrome.
pubmed:affiliation
Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands. m.g.duffels@amc.uva.nl
pubmed:publicationType
Journal Article, Comparative Study, Evaluation Studies