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Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation: The TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) Study.

Hebert, Anders ; Mikkelsen, Ulla R ; Thilén, Ulf LU ; Idorn, Lars ; Jensen, Annette S ; Nagy, Edit ; Hanséus, Katarina LU ; Sørensen, Keld E and Søndergaard, Lars (2014) In Circulation 130(23). p.2021-2030
Abstract
The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients.
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author
; ; ; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Circulation
volume
130
issue
23
pages
2021 - 2030
publisher
Lippincott Williams & Wilkins
external identifiers
  • pmid:25446057
  • wos:000346033700012
  • scopus:84916631939
  • pmid:25446057
ISSN
1524-4539
DOI
10.1161/CIRCULATIONAHA.113.008441
language
English
LU publication?
yes
id
ae6b1264-70ac-4b7c-a76b-a38b8692fff8 (old id 4913389)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/25446057?dopt=Abstract
date added to LUP
2016-04-01 10:37:18
date last changed
2022-04-27 23:43:18
@article{ae6b1264-70ac-4b7c-a76b-a38b8692fff8,
  abstract     = {{The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients.}},
  author       = {{Hebert, Anders and Mikkelsen, Ulla R and Thilén, Ulf and Idorn, Lars and Jensen, Annette S and Nagy, Edit and Hanséus, Katarina and Sørensen, Keld E and Søndergaard, Lars}},
  issn         = {{1524-4539}},
  language     = {{eng}},
  number       = {{23}},
  pages        = {{2021--2030}},
  publisher    = {{Lippincott Williams & Wilkins}},
  series       = {{Circulation}},
  title        = {{Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation: The TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) Study.}},
  url          = {{http://dx.doi.org/10.1161/CIRCULATIONAHA.113.008441}},
  doi          = {{10.1161/CIRCULATIONAHA.113.008441}},
  volume       = {{130}},
  year         = {{2014}},
}