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.

Anders Hebert; Ulla R Mikkelsen; Ulf Thilén; Lars Idorn; Annette S Jensen; Edit Nagy; Katarina Hanséus; Keld E Sørensen; Lars Søndergaard

doi:10.1161/CIRCULATIONAHA.113.008441

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.

Anders Hebert, Ulla R Mikkelsen, Ulf Thilén, Lars Idorn, Annette S Jensen, Edit Nagy, Katarina Hanséus, Keld E Sørensen, Lars Søndergaard

Research output: Contribution to journal › Article › peer-review

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.

Original language	English
Pages (from-to)	2021-2030
Journal	Circulation
Volume	130
Issue number	23
DOIs	https://doi.org/10.1161/CIRCULATIONAHA.113.008441
Publication status	Published - 2014

Subject classification (UKÄ)

Cardiology and Cardiovascular Disease

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10.1161/CIRCULATIONAHA.113.008441

http://www.ncbi.nlm.nih.gov/pubmed/25446057?dopt=Abstract

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Hebert, A., Mikkelsen, U. R., Thilén, U., Idorn, L., Jensen, A. S., Nagy, E., Hanséus, K., Sørensen, K. E., & Søndergaard, L. (2014). 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. Circulation, 130(23), 2021-2030. https://doi.org/10.1161/CIRCULATIONAHA.113.008441

Hebert, Anders ; Mikkelsen, Ulla R ; Thilén, Ulf et al. / 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. In: Circulation. 2014 ; Vol. 130, No. 23. pp. 2021-2030.

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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.",

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 = "Anders Hebert and Mikkelsen, {Ulla R} and Ulf Thil{\'e}n and Lars Idorn and Jensen, {Annette S} and Edit Nagy and Katarina Hans{\'e}us and S{\o}rensen, {Keld E} and Lars S{\o}ndergaard",

year = "2014",

doi = "10.1161/CIRCULATIONAHA.113.008441",

language = "English",

volume = "130",

pages = "2021--2030",

journal = "Circulation",

issn = "1524-4539",

publisher = "Lippincott Williams & Wilkins",

number = "23",

}

Hebert, A, Mikkelsen, UR, Thilén, U, Idorn, L, Jensen, AS, Nagy, E, Hanséus, K, Sørensen, KE & Søndergaard, L 2014, '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.', Circulation, vol. 130, no. 23, pp. 2021-2030. https://doi.org/10.1161/CIRCULATIONAHA.113.008441

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 et al.
In: Circulation, Vol. 130, No. 23, 2014, p. 2021-2030.

Research output: Contribution to journal › Article › peer-review

TY - JOUR

T1 - 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.

AU - Hebert, Anders

AU - Mikkelsen, Ulla R

AU - Thilén, Ulf

AU - Idorn, Lars

AU - Jensen, Annette S

AU - Nagy, Edit

AU - Hanséus, Katarina

AU - Sørensen, Keld E

AU - Søndergaard, Lars

PY - 2014

Y1 - 2014

N2 - 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.

AB - 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.

U2 - 10.1161/CIRCULATIONAHA.113.008441

DO - 10.1161/CIRCULATIONAHA.113.008441

M3 - Article

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SN - 1524-4539

VL - 130

SP - 2021

EP - 2030

JO - Circulation

JF - Circulation

IS - 23

ER -

Hebert A, Mikkelsen UR, Thilén U, Idorn L, Jensen AS, Nagy E et al. 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. Circulation. 2014;130(23):2021-2030. doi: 10.1161/CIRCULATIONAHA.113.008441