PMID- 23440179
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20130227
LR  - 20240322
IS  - 1179-5468 (Print)
IS  - 1179-5468 (Electronic)
IS  - 1179-5468 (Linking)
VI  - 7
DP  - 2013
TI  - Effect of bosentan on exercise capacity and clinical worsening in patients with 
      dual down and eisenmenger syndrome.
PG  - 29-34
LID - 10.4137/CMC.S10237 [doi]
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.
FAU - Serino, Giorgio
AU  - Serino G
AD  - Department of Internal and Vascular Medicine, IRCCS-Policlinico, San Donato, 
      Italy.
FAU - Guazzi, Marco
AU  - Guazzi M
FAU - Micheletti, Angelo
AU  - Micheletti A
FAU - Lombardi, Carlo
AU  - Lombardi C
FAU - Danesi, Rossella
AU  - Danesi R
FAU - Negura, Diana
AU  - Negura D
FAU - Carminati, Mario
AU  - Carminati M
FAU - Chessa, Massimo
AU  - Chessa M
LA  - eng
PT  - Journal Article
DEP - 20130206
PL  - United States
TA  - Clin Med Insights Cardiol
JT  - Clinical Medicine Insights. Cardiology
JID - 101525768
PMC - PMC3572875
OTO - NOTNLM
OT  - Down syndrome
OT  - Eisenmenger syndrome
OT  - bosentan
OT  - congenital heart disease
OT  - endothelin receptor antagonist
OT  - pulmonary arterial hypertension
EDAT- 2013/02/27 06:00
MHDA- 2013/02/27 06:01
PMCR- 2013/02/06
CRDT- 2013/02/27 06:00
PHST- 2013/02/27 06:00 [entrez]
PHST- 2013/02/27 06:00 [pubmed]
PHST- 2013/02/27 06:01 [medline]
PHST- 2013/02/06 00:00 [pmc-release]
AID - cmc-7-2013-029 [pii]
AID - 10.4137/CMC.S10237 [doi]
PST - ppublish
SO  - Clin Med Insights Cardiol. 2013;7:29-34. doi: 10.4137/CMC.S10237. Epub 2013 Feb 
      6.