PULMONARY HYPERTENSION

Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease

Gerhard-Paul Diller¹, Konstantinos Dimopoulos¹, Mehmet G Kaya¹, Carl Harries¹, Anselm Uebing², Wei Li¹, Evdokia Koltsida¹, J Simon R Gibbs³ and Michael A Gatzoulis¹

¹ Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, and the National Heart and Lung Institute, Imperial College of Science and Medicine, London, UK
² Department of Paediatric Cardiology and Biomedical Engineering, University Hospital of Schleswig-Holstein, Kiel, Germany
³ Department of Cardiology, Hammersmith Hospital, London, UK

Correspondence to:
Correspondence to:
Professor M A Gatzoulis
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; m.gatzoulis{at}rbh.nthames.nhs.uk

Objective: To examine long-term safety and efficacy of bosentan—an oral dual endothelin receptor antagonist—in patients with pulmonary hypertension associated with congenital heart disease or Eisenmenger’s syndrome.

Design: Retrospective study.

Setting: Tertiary cardiology referral centre.

Patients: All adult patients with pulmonary arterial hypertension associated with congenital heart disease treated with bosentan at the Royal Brompton Adult Congenital Heart Centre were included.

Main outcome measures: Oxygen saturation, functional (WHO) class, 6-minute walk test distance and liver enzymes were analysed.

Results: Eighteen patients (14 female) with pulmonary arterial hypertension associated with congenital heart disease (15 patients with Eisenmenger’s syndrome) with a mean (SD) age of 41 (9) years (range 23–69) were included. Median follow-up was 29 months (range 1–39). One patient died during follow-up. Patients tolerated bosentan well and no significant rise in liver transaminases was seen. Arterial oxygen saturation remained stable throughout follow-up. Mean (SD) functional class (p = 0.001) and the 6-minute walk test distance improved compared with baseline (284 (144) vs 363 (124) m, 380 (91) m and 408 (114) m at baseline, 0–6 months, 6–12 months and 1–2 years of treatment, respectively; p<0.05 for each).

Conclusions: Bosentan appears to be safe and well tolerated in adults with pulmonary arterial hypertension associated with congenital heart disease or Eisenmenger’s syndrome during mid- to long-term follow-up. In addition, functional class and the 6-minute walk test distance improved and this effect was maintained for up to 2 years of bosentan treatment.

Abbreviations: PAH, pulmonary arterial hypertension; SMWTd, 6-minute walk test distance; VSD, ventricular septal defect

Keywords: bosentan; congenital heart disease; Eisenmenger’s syndrome; endothelin antagonism; pulmonary arterial hypertension

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Duffels, M. G.J., Hardziyenka, M., Surie, S., de Bruin-Bon, R. H.A.C.M, Hoendermis, E. S., van Dijk, A. P.J., Bouma, B. J., Tan, H. L., Berger, R. M.F., Bresser, P., Mulder, B. J.M. (2009). Duration of right ventricular contraction predicts the efficacy of bosentan treatment in patients with pulmonary hypertension. Eur J Echocardiogr 10: 433-438 [Abstract] [Full Text]  
• Beghetti, M., Galie, N. (2009). Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension.. J Am Coll Cardiol 53: 733-740 [Abstract] [Full Text]  
• Bedard, E., Dimopoulos, K., Gatzoulis, M. A. (2009). Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension?. Eur Heart J 30: 256-265 [Abstract] [Full Text]  
• National Pulmonary Hypertension Centres of the UK, (2008). Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Heart 94: i1-i41 [Full Text]  
• National Pulmonary Hypertension Centres of the UK, (2008). Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Thorax 63: ii1-ii41 [Full Text]