Abstract
This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.
| Original language | English |
|---|---|
| Pages (from-to) | 29-34 |
| Number of pages | 6 |
| Journal | Clinical Medicine Insights: Cardiology |
| Volume | 7 |
| DOIs | |
| Publication status | Published - 2013 |
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Keywords
- Bosentan
- Congenital heart disease
- Down syndrome
- Eisenmenger syndrome
- Endothelin receptor antagonist
- Pulmonary arterial hypertension
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
Cite this
Effect of bosentan on exercise capacity and clinical worsening in patients with dual Down and Eisenmenger syndrome. / Serino, Giorgio; Guazzi, Marco; Micheletti, Angelo; Lombardi, Carlo; Danesi, Rossella; Negura, Diana; Carminati, Mario; Chessa, Massimo.
In: Clinical Medicine Insights: Cardiology, Vol. 7, 2013, p. 29-34.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Effect of bosentan on exercise capacity and clinical worsening in patients with dual Down and Eisenmenger syndrome
AU - Serino, Giorgio
AU - Guazzi, Marco
AU - Micheletti, Angelo
AU - Lombardi, Carlo
AU - Danesi, Rossella
AU - Negura, Diana
AU - Carminati, Mario
AU - Chessa, Massimo
PY - 2013
Y1 - 2013
N2 - This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.
AB - This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.
KW - Bosentan
KW - Congenital heart disease
KW - Down syndrome
KW - Eisenmenger syndrome
KW - Endothelin receptor antagonist
KW - Pulmonary arterial hypertension
UR - http://www.scopus.com/inward/record.url?scp=84873895415&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84873895415&partnerID=8YFLogxK
U2 - 10.4137/CMC.S10237
DO - 10.4137/CMC.S10237
M3 - Article
C2 - 23440179
AN - SCOPUS:84873895415
VL - 7
SP - 29
EP - 34
JO - Clinical Medicine Insights: Cardiology
JF - Clinical Medicine Insights: Cardiology
SN - 1179-5468
ER -