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The effect of bosentan in patients with a failing Fontan circulation

Published online by Cambridge University Press:  01 August 2009

Caroline Ovaert ,
Daisy Thijs ,
Daniel Dewolf ,
Jaap Ottenkamp ,
Hugues Dessy ,
Philip Moons ,
Marc Gewillig  and
Luc Mertens
Caroline Ovaert
Affiliation:
Department of Pediatric Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
Daisy Thijs
Affiliation:
Department of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium
Daniel Dewolf
Affiliation:
Department of Pediatric Cardiology, Universitair Ziekenhuis Gent, Gent, Belgium
Jaap Ottenkamp
Affiliation:
Department of Pediatric Cardiology, University Medical Centre, Leiden, Netherlands
Hugues Dessy
Affiliation:
Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, Brussels, Belgium
Philip Moons
Affiliation:
Center for Health Services and Nursing Research, University Hospitals Leuven, Leuven, Belgium
Marc Gewillig
Affiliation:
Department of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium
Luc Mertens*
Affiliation:
Department of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium
*
Correspondence to: Luc Mertens, MD, PhD, The Hospital for Sick Children, The Labatt Family Heart Centre, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. Tel: +1-416-813-7291; Fax: +1-416-813-5857; E-mail: luc.mertens@sickkids.ca
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Abstract

Objectives

To investigate the effect of bosentan in patients with a failing Fontan circulation.

Design

A multicentric open label, non-controlled study.

Setting

5 tertiary care centres for congenital cardiology.

Patients

We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks.

Main measures of outcomes

We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded.

Results

Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued.

Conclusions

Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.

Keywords

Congenital heart defectfunctionally single ventricleendothelin receptor antagonistpulmonary vascular resistance
Type
Original Articles
Information
Cardiology in the Young , Volume 19 , Issue 4 , August 2009 , pp. 331 - 339
Copyright
Copyright © Cambridge University Press 2009

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