Endothelin receptor antagonists are an effective long term treatment option in pulmonary arterial hypertension associated with congenital heart disease with or without Trisomy 21

Kermeen, F.D., Franks, C., O'Brien, K., Seale, H., Hall, K., McNeil, K. and Radford, D. (2010) Endothelin receptor antagonists are an effective long term treatment option in pulmonary arterial hypertension associated with congenital heart disease with or without Trisomy 21. Heart, Lung and Circulation, 19 10: 595-600. doi:10.1016/j.hlc.2010.07.005


Author Kermeen, F.D.
Franks, C.
O'Brien, K.
Seale, H.
Hall, K.
McNeil, K.
Radford, D.
Title Endothelin receptor antagonists are an effective long term treatment option in pulmonary arterial hypertension associated with congenital heart disease with or without Trisomy 21
Journal name Heart, Lung and Circulation   Check publisher's open access policy
ISSN 1443-9506
Publication date 2010-10
Sub-type Article (original research)
DOI 10.1016/j.hlc.2010.07.005
Volume 19
Issue 10
Start page 595
End page 600
Total pages 6
Place of publication Chatswood, NSW, Australia
Publisher Elsevier Australia
Language eng
Abstract Introduction: Traditionally, treatment options for patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) are limited. Bosentan has been shown to improve pulmonary haemodynamics and exercise tolerance short term but long term clinical studies are lacking. Aim: To report long term efficacy and safety data with endothelin receptor antagonists (ERA) in patients with PAH associated CHD. Methods: Prospective, open label, uncontrolled, single centre study of 53 patients (33 females, 17 Trisomy 21, mean age 34 ± 12 years) prescribed ERA (48 bosentan, 5 sitaxentan) from 2003 to August 2009. Outcome measurements of oxygen saturation (SaO2), WHO functional class, 6-minute walk test distance (6MWD) and adverse events were analysed. Results: Mean duration of therapy was 15 ± 13 months in 53 patients with CHD. Four patients failed ERA, seven died (five progressive RHF) and one delisted from transplantation. No abnormal liver transaminases occurred on bosentan, with one case on sitaxentan. After 3, 6, 12, 18 and 24 months of treatment a significant improvement was seen in WHO functional class (mean 3.15 vs 2.8 vs 2.5 vs 2.5 vs 2.4 vs 2.4; p<0.01) and 6MWD (344 ± 18 vs 392 ± 17 vs 411 ± 17 vs 420 ± 17 vs 442 ± 18 vs 417 ± 23: p<0.0005, p<0.01) compared with baseline. The Trisomy 21 and PAH-CHD showed a significant improvement in 6MWD at 6 and 12 months (263 ± 24 vs 348 ± 29 vs 360 ± 32, p< 0.01, p< 0.05) respectively. No changes in SaO2, BNP, RV or LV function were demonstrated during follow-up. Conclusion: This large single centre study demonstrates that endothelin receptor antagonism is an effective and safe treatment in PAH associated CHD with or without Trisomy 21. The improvements in exercise tolerance are similar to reported benefits in other forms of PAH. Crown Copyright © 2010 Published by Elsevier Inc. on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. All rights reserved.
Keyword Eisenmenger syndrome
Endothelin receptor antagonists
Trisomy 21
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Unknown

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
 
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Created: Wed, 16 Mar 2011, 14:57:46 EST by Debbie Banks on behalf of School of Medicine