Skip to main content
SpringerLink
Log in

Bosentan in pulmonary arterial hypertension: a comparison between congenital heart disease and chronic pulmonary embolism

  • Original article
  • Published:
Netherlands Heart Journal Aims and scope Submit manuscript

Abstract

Background. In patients with pulmonary hypertension, it is unknown whether the treatment effect of bosentan is dependent on the duration of pulmonary vessel changes. Therefore, we studied the response to bosentan in patients with life-long pulmonary vessel changes (pulmonary arterial hypertension (PAH) due to congenital heart disease (CHD)) and in patients with subacutely induced pulmonary vessel changes (chronic thromboembolic pulmonary hypertension (CTEPH)).

Methods. In this open-label study, 18 patients with PAH due to CHD and 16 patients with CTEPH were treated with bosentan for at least one year. All patients were evaluated at baseline and during follow-up by means of the six-minute walk distance (6-MWD) and laboratory tests.

Results. Improvement of 6-MWD was comparable in patients with PAH due to CHD (444±112 m to 471±100 m, p=0.02), and in CTEPH (376±152 m to 423±141 m, p=0.03) after three months of treatment. After this improvement, 6-MWD stabilised in both groups.

Conclusion. Although duration of pulmonary vessel changes is strikingly different in patients with PAH due to CHD and CTEPH, the effect of one year of bosentan treatment was comparable. The main treatment effect appears to be disease stabilisation and decreasing the rate of deterioration. (Neth Heart J 2009;17:334–8.)

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43:5S–12S.

    Google Scholar 

  2. Engelfriet PM, Duffels MG, Moller T, Boersma E, Tijssen JG, Thaulow E, et al. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007;93:682–7.

    Google Scholar 

  3. Riedel M, Stanek V, Widimsky J, Prerovsky I. Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest. 1982;81: 151–8.

    Google Scholar 

  4. Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, Vriend JW, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007;120:198–204.

    Google Scholar 

  5. Oya H, Nagaya N, Uematsu M, Satoh T, Sakamaki F, Kyotani S, et al. Poor prognosis and related factors in adults with Eisenmenger syndrome. Am Heart J. 2002;143:739–44.

    Google Scholar 

  6. Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2001;345:1465–72.

    Google Scholar 

  7. Pengo V, Lensing AW, Prins M, Prins MH, Marchiori A, Marchiori A, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350:2257–64.

    Google Scholar 

  8. Bresser P, Pepke-Zaba J, Jais X, Humbert M, Hoeper MM. Medical therapies for chronic thromboembolic pulmonary hypertension: an evolving treatment paradigm. Proc Am Thorac Soc. 2006;3:594–600.

    Google Scholar 

  9. Auger WR, Kerr KM, Kim NH, Ben-Yehuda O, Knowlton KU, Fedullo PF. Chronic thromboembolic pulmonary hypertension. Cardiol Clin. 2004;22:453–66, vii.

    Google Scholar 

  10. Bonderman D, Nowotny R, Skoro-Sajer N, Jakowitsch J, Adlbrecht C, Klepetko W, et al. Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest. 2005; 128:2599–603.

    Google Scholar 

  11. Apostolopoulou SC, Manginas A, Cokkinos DV, Rammos S. Long-term oral bosentan treatment in patients with pulmonary arterial hypertension related to congenital heart disease: a 2-year study. Heart. 2007;93:350–4.

    Google Scholar 

  12. D'Alto M, Vizza CD, Romeo E, Badagliacca R, Santoro G, Poscia R, et al. Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect. Heart. 2007;93:621–5.

    Google Scholar 

  13. Hoeper MM, Kramm T, Wilkens H, Schulze C, Schafers HJ, Welte T, et al. Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest. 2005;128:2363–7.

    Google Scholar 

  14. Gatzoulis MA, Beghetti M, Galie N, Granton J, Berger RM, Lauer A, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: Results of the BREATHE-5 open-label extension study. Int J Cardiol. 2007;127:27–32.

    Google Scholar 

  15. Galie N, Beghetti M, Gatzoulis MA, Granton J, Berger RMF, Lauer A, et al. Bosentan therapy in patients with Eisenmenger syndrome: A multicenter, double-blind, randomized, placebocontrolled study. Circulation. 2006;114:48–54.

    Google Scholar 

  16. Rubin LJ, Badesch DB. Evaluation and management of the patient with pulmonary arterial hypertension. Ann Intern Med. 2005;143: 282–92.

    Google Scholar 

  17. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med. 2002;166:111–7.

    Google Scholar 

  18. Apostolopoulou SC, Manginas A, Cokkinos DV, Rammos S. Effect of the oral endothelin antagonist bosentan on the clinical, exercise, and haemodynamic status of patients with pulmonary arterial hypertension related to congenital heart disease. Heart. 2005;91:1447–52.

    Google Scholar 

  19. Duffels MGJ, Berger RMF, Bresser P, de Bruin-Bon HACM, Hoendermis E, Bouma BJ, et al. Applicability of bosentan in Dutch patients with Eisenmenger syndrome: preliminary results on safety and exercise capacity. Neth Heart J. 2006;14:165–70.

    Google Scholar 

  20. Duffels M, van Loon L, Berger R, Boonstra A, Vonk-Noordergraaf A, Mulder B. Pulmonary arterial hypertension associated with a congenital heart defect: advanced medium-term medical treatment stabilizes clinical condition. Congenit Heart Dis. 2007;2:242–9.

    Google Scholar 

  21. Seyfarth HJ, Hammerschmidt S, Pankau H, Winkler J, Wirtz H. Long-term bosentan in chronic thromboembolic pulmonary hypertension. Respiration. 2007;74:287–92.

    Google Scholar 

  22. Diller GP, Dimopoulos K, Kaya MG, Harries C, Uebing A, Li W, et al. Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart. 2007;93:974–6.

    Google Scholar 

  23. van Loon RL, Hoendermis ES, Duffels MG, Vonk-Noordegraaf A, Mulder BJ, Hillege HL, et al. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist? Am Heart J. 2007;154:776–82.

    Google Scholar 

  24. Hughes RJ, Jais X, Bonderman D, Suntharalingam J, Humbert M, Lang I, et al. The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study. Eur Respir J. 2006;28:138–43.

    Google Scholar 

  25. McLaughlin VV, Presberg KW, Doyle RL, Abman SH, McCrory DC, Fortin T, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004; 126:78S–92S.

    Google Scholar 

  26. Hopkins WE, Waggoner AD. Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome. Am J Cardiol.. 2002;89:34–8.

    Google Scholar 

  27. Enright PL, Sherrill DL. Reference equations for the six-minute walk in healthy adults. Am J Respir Crit Care Med. 1998;158: 1384–7.

    Google Scholar 

  28. Ulrich S, Fischler M, Speich R, Popov V, Maggiorini M. Chronic thromboembolic and pulmonary arterial hypertension share acute vasoreactivity properties. Chest. 2006;130:841–6.

    Google Scholar 

  29. Cenedese E, Speich R, Dorschner L, Ulrich S, Maggiorini M, Jenni R, et al. Measurement of quality of life in pulmonary hypertension and its significance. Eur Respir J. 2006;28:808–15.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. www.bsl.nl/shop/

    M. G. J. Duffels

Authors
  1. M. G. J. Duffels
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. N. van der Plas
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. S. Surie
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M. M. Winter
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. B. J. Bouma
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. M. Groenink
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. A. P. J. van Dijk
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. E. S. Hoendermis
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. R. M. F. Berger
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. P. Bresser
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. B. J. M. Mulder
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands

Department of Pulmonology, Academic Medical Center, Amsterdam, the Netherlands

Department of Cardiology, University Medical Center Nijmegen, the Netherlands

Department of Cardiology, University Medical Center Groningen, the Netherlands

Department of Paediatric Cardiology University Medical Center Groningen, the Netherlands

Departments of Cardiology, Academic Medical Center, Amsterdam and University Medical Center Utrecht, Utrecht, the Netherlands

B.J.M. Mulder Department of Cardiology, Rm B2–240, Academic Medical Center, PO Box 22660, 1100 DD Amsterdam, the Netherlands

Rights and permissions

Reprints and permissions

About this article

Cite this article

Duffels, M.G.J., van der Plas, M.N., Surie, S. et al. Bosentan in pulmonary arterial hypertension: a comparison between congenital heart disease and chronic pulmonary embolism. NHJL 17, 334–338 (2009). https://doi.org/10.1007/BF03086279

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03086279

Search

Navigation