The effect of Bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study

A Hebert, AS Jensen, L Idorn, KE Sørensen… - BMC Cardiovascular …, 2013 - Springer
Background Palliative treatment with the Fontan procedure has greatly improved survival for
children with functionally univentricular heart. Since Fontan performed the first successful …

Rationale and design of a trial on the role of bosentan in Fontan patients: improvement of exercise capacity?

MJ Schuuring, JC Vis, BJ Bouma, APJ van Dijk… - Contemporary clinical …, 2011 - Elsevier
Abstract Background The Fontan circulation is a palliative procedure performed in patients
with complex congenital heart disease (CHD), making transpulmonary blood flow …

Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (treatment with endothelin receptor antagonist in Fontan patients, a …

A Hebert, UR Mikkelsen, U Thilen, L Idorn, AS Jensen… - Circulation, 2014 - Am Heart Assoc
Background—The Fontan procedure has improved survival in children with functionally
univentricular hearts. With time, however, complications such as reduced exercise capacity …

Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial

MJ Schuuring, JC Vis, APJ van Dijk… - European journal of …, 2013 - Wiley Online Library
Aims An endothelin‐1 receptor blocker, shown to be effective in patients with pulmonary
arterial hypertension, might decrease pulmonary vascular resistance to increase cardiac …

[HTML][HTML] Pharmacology management in improving exercise capacity of patients with fontan circulation: a systematic review and meta-analysis

B Mendel, M Setiawan, SN Siagian… - Current Cardiology …, 2022 - ncbi.nlm.nih.gov
Background: The Fontan procedure is currently the mainstay therapy for single functional
ventricles. However, with prolonged follow-up duration, various complications have been …

Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy

BS Snarr, SM Paridon, J Rychik… - Cardiology in the …, 2015 - cambridge.org
The Fontan operation is the final step of palliation for patients with a functionally single
ventricle. Since its introduction in the 1970s, the Fontan surgery has become part of a …

Effect of ambrisentan on exercise capacity in adult patients after the Fontan procedure

AM Cedars, J Saef, LR Peterson, AR Coggan… - The American Journal of …, 2016 - Elsevier
The Fontan operation is a common end point for children born with a single functional
ventricle. Fontan patients typically experience physiological deterioration leading to …

Managing adult Fontan patients: where do we stand?

P Clift, D Celermajer - European Respiratory Review, 2016 - Eur Respiratory Soc
The Fontan operation is performed as a palliative procedure to improve survival in infants
born with a functionally univentricular circulation. The success of the operation is …

The efficacy and safety of macitentan in Fontan-palliated patients: results of the 52-week randomised, placebo-controlled RUBATO trial

P Clift, F Berger, L Sondergaard… - European Heart …, 2022 - academic.oup.com
Background The clinical utility and long-term effects of endothelin receptor antagonists
(ERAs) in Fontan-palliated patients remain unclear and there are currently no approved …

The safety and effects of bosentan in patients with a Fontan circulation

SE Bowater, RA Weaver, SA Thorne… - Congenital heart …, 2012 - Wiley Online Library
Objective. Adult patients with a Fontan circulation tend to have diminished exercise capacity.
The principal objective of this study was to investigate the safety of the endothelin receptor …