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Bosentan for the treatment of pulmonary arterial hypertension associated with congenital cardiac disease

Published online by Cambridge University Press:  26 May 2006

Eugene Kotlyar ,
Raymond Sy ,
Anne M. Keogh ,
Fiona Kermeen ,
Peter S. Macdonald ,
Christopher S. Hayward ,
Keith D. McNeil  and
David S. Celermajer
Eugene Kotlyar
Affiliation:
St Vincent's Hospital, Sydney, Australia
Raymond Sy
Affiliation:
Royal Prince Alfred Hospital, Sydney, Australia
Anne M. Keogh
Affiliation:
St Vincent's Hospital, Sydney, Australia
Fiona Kermeen
Affiliation:
Prince Charles Hospital, Brisbane, Australia
Peter S. Macdonald
Affiliation:
St Vincent's Hospital, Sydney, Australia
Christopher S. Hayward
Affiliation:
St Vincent's Hospital, Sydney, Australia
Keith D. McNeil
Affiliation:
Prince Charles Hospital, Brisbane, Australia
David S. Celermajer
Affiliation:
Royal Prince Alfred Hospital, Sydney, Australia
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Abstract

Aims: Bosentan is efficacious in idiopathic pulmonary arterial hypertension, and the variants associated with connective tissue disease, but not currently approved for treatment of pulmonary arterial hypertension due to Eisenmenger's syndrome. We sought to evaluate its effect in adults with Eisenmenger's syndrome. Methods: We administered bosentan on the basis of compassionate use in 23 patients with Eisenmenger's syndrome, aged 37 plus or minus 14 years. Of the patients, 17 had never received specific treatment for pulmonary arterial hypertension, five were transitioned from treprostinil, and one from beraprost to bosentan. We measured functional class, saturation of oxygen, haemoglobin levels and six-minute walk distance at baseline, one, six months and at most recent follow-up. Results: Baseline functional class was IV in three, III in fifteen, and II in five patients. At follow-up, with a mean of 15 plus or minus 10 months, 13 of the 23 patients (57%) had improved by at least one functional class, from a median baseline of III to II (p equal to 0.016), mean saturation of oxygen at rest had increased from 81% to 84% (p equal to 0.001), and levels of haemoglobin had decreased from 178 plus or minus 26 grams per litre to 167 plus or minus 19 grams per litre (p equal to 0.001). Overall, the six-minute walk distance did not change from baseline of 335 metres. The distance walked by those not previously receiving specific therapy, however, improved from 318 plus or minus 129 to 345 plus or minus 123 metres (p equal to 0.03). Conclusion: Treatment of adults with Eisenmenger's syndrome using bosentan significantly improved functional class, saturation of oxygen at rest, and decreased levels of haemoglobin. Treatment with bosentan was associated with improvement in six-minute walk distance in those not previously receiving specific therapy. In patients already in receipt of specific therapy, transition to bosentan resulted in no clinical deterioration.

Keywords

Endothelin antagonistscyanotic heart diseaseEisenmenger syndrome
Type
Original Article
Information
Cardiology in the Young , Volume 16 , Issue 3 , June 2006 , pp. 268 - 274
Copyright
© 2006 Cambridge University Press

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