Why the study?
Does Bosentan improve symptoms, oxygen saturation, and exercise capacity in pediatric patients with Eisenmenger's syndrome?
Does Bosentan improve symptoms, oxygen saturation, and exercise capacity in pediatric patients with Eisenmenger's syndrome?
Bosentan provides symptomatic improvement in pediatric patients with Eisenmenger's syndrome over 12 months, despite no improvement in oxygen saturation and worsening exercise capacity.
Symptomatic benefit with bosentan in pediatric Eisenmenger syndrome was unaccompanied by oxygenation or exercise gains; leaves open efficacy pending controlled trials.
We treated prospectively 14 patients with Eisenmenger's syndrome, with a mean age of 10 years, ranging from 3 to 18 years. Treatment continued for 12 months, and demonstrated a lasting symptomatic improvement, but no improvement in terms of mean saturation of oxygen over 24 hours. Exercise capacity, as judged by peak uptake of oxygen, worsened in the six patients able to perform a treadmill test. The symptomatic benefit from dual blockage of endothelin receptors in these patients may be due to mechanisms other than selective pulmonary vasodilatation alone.
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Brun et al. (2007) studied this question.
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