Document Detail

Rationale and design of a trial on the role of bosentan in Fontan patients: improvement of exercise capacity?
MedLine Citation:
PMID:  21527360     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: The Fontan circulation is a palliative procedure performed in patients with complex congenital heart disease (CHD), making transpulmonary blood flow dependent on the systemic venous pressure. In a Fontan circulation a low pulmonary vascular resistance (PVR) is crucial, as is epitomized by the observation that a high PVR is a strong predictor of mortality. Long-term follow-up has shown that PVR may rise many years after the Fontan procedure has been performed, possibly due to micro-emboli from a dilated right atrium or from the venous system. Other mechanisms of increased PVR might be aging, obstructed airways caused by lymphatic dysfunction, lack of pulsatile pulmonary flow causing a release of endothelium-derived vasoactive molecules, and prolonged overexpression of vasoconstrictors such as endothelin-1. Mean plasma level of endothelin-1 has been shown to be significantly higher in Fontan patients compared to healthy controls. In patients with pulmonary arterial hypertension (PAH), therapy with bosentan, an endothelin-1 receptor antagonist, has demonstrated to improve exercise capacity and to reduce the elevated PVR. In addition, reduction of PVR is shown early and late after the Fontan procedure on treatment with exogenous NO, another advanced PAH therapy. However, the long term effect of reducing the PVR by bosentan treatment on exercise capacity in Fontan patients is still unknown.
METHODS: We designed a prospective, multicenter, randomized open label trial to study the effect of bosentan in Fontan patients. The primary endpoint will be the change in maximum exercise capacity (peak V'O2).
CONCLUSION: We hypothesize that treatment with bosentan, an endothelin-1 receptor antagonist, improves maximum exercise capacity and functional capacity in adult Fontan patients.
Mark J Schuuring; Jeroen C Vis; Berto J Bouma; Arie P J van Dijk; Joost P van Melle; Petronella G Pieper; Hubert W Vliegen; Gertjan Tj Sieswerda; Barbara J M Mulder
Publication Detail:
Type:  Journal Article; Multicenter Study; Randomized Controlled Trial     Date:  2011-04-17
Journal Detail:
Title:  Contemporary clinical trials     Volume:  32     ISSN:  1559-2030     ISO Abbreviation:  Contemp Clin Trials     Publication Date:  2011 Jul 
Date Detail:
Created Date:  2011-05-30     Completed Date:  2011-11-08     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  101242342     Medline TA:  Contemp Clin Trials     Country:  United States    
Other Details:
Languages:  eng     Pagination:  586-91     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 Elsevier Inc. All rights reserved.
Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands.
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MeSH Terms
Antihypertensive Agents / pharmacology*
Clinical Protocols
Exercise Test
Exercise Tolerance / drug effects*
Fontan Procedure*
Oxygen Consumption / drug effects*
Patient Selection
Prospective Studies
Random Allocation
Research Design*
Sulfonamides / pharmacology*
Reg. No./Substance:
0/Antihypertensive Agents; 0/Sulfonamides; Q326023R30/bosentan

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