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.

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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.


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

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Kardiologi
Sidor (från-till)2021-2030
Utgåva nummer23
StatusPublished - 2014
Peer review utfördJa