Hostname: page-component-cd9895bd7-8ctnn Total loading time: 0 Render date: 2024-12-24T14:11:17.705Z Has data issue: false hasContentIssue false
  • English
  • Français

Treatment of patients with Eisenmenger's syndrome with Bosentan

Published online by Cambridge University Press:  23 April 2007

Henrik Brun ,
Erik Thaulow ,
Per Morten Fredriksen  and
Henrik Holmstrom
Henrik Brun
Affiliation:
Paediatric Cardiology Unit, Rikshospitalet, Oslo, Norway
Erik Thaulow
Affiliation:
Paediatric Cardiology Unit, Rikshospitalet, Oslo, Norway
Per Morten Fredriksen
Affiliation:
Physiotherapy Department, Rikshospitalet, Oslo, Norway
Henrik Holmstrom
Affiliation:
Paediatric Cardiology Unit, Rikshospitalet, Oslo, Norway
Get access Rights & Permissions [Opens in a new window]

Abstract

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.

Keywords

Pulmonary hypertensioncongenital heart diseaseexercise testing
Type
Original Article
Information
Cardiology in the Young , Volume 17 , Issue 3 , June 2007 , pp. 288 - 294
Copyright
© 2007 Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001; 358: 1119–1123.Google Scholar
Sitbon O, Badesch DB, Channick RN, et al. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest 2003; 124: 247–254.Google Scholar
Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896–903.Google Scholar
McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005; 25: 244–249.Google Scholar
Rabinovitch M, Haworth SG, Castaneda AR, Nadas AS, Reid LM. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation 1978; 58: 1107–1122.Google Scholar
Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome – Factors relating to deterioration and death. Eur Heart J 1998; 19: 1845–1855.Google Scholar
Hopkins WE, Ochoa LL, Richardson GW, Trulock EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant 1996; 15(1 Pt 1): 100–105.Google Scholar
Hopkins WE, Waggoner AD. Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome. Am J Cardiol 2002; 89: 34–38.Google Scholar
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: 1447–1452.Google Scholar
Christensen DD, McConnell ME, Book WM, Mahle WT. Initial experience with bosentan therapy in patients with the Eisenmenger syndrome. Am J Cardiol 2004; 94: 261–263.Google Scholar
Gatzoulis MA, Rogers P, Li W, et al. Safety and tolerability of bosentan in adults with Eisenmenger physiology. Int J Cardiol 2005; 98: 147–151.Google Scholar
Schulze-Neick I, Gilbert N, Ewert R, et al. Adult patients with congenital heart disease and pulmonary arterial hypertension: first open prospective multicenter study of bosentan therapy. Am Heart J 2005; 150: 716.Google Scholar
Barst RJ, Ivy D, Dingemanse J, et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther 2003; 73: 372–382.Google Scholar
Rosenzweig EB, Ivy DD, Widlitz A, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005; 46: 697–704.Google Scholar
Bowyer JJ, Busst CM, Denison DM, Shinebourne EA. Effect of long-term oxygen treatment at home in children with pulmonary vascular disease. Br Heart J 1986; 55: 385–390.Google Scholar
Fredriksen PM, Ingjer F, Nystad W, Thaulow E. Aerobic endurance testing of children and adolescents – a comparison of two treadmill-protocols. Scand J Med Sci Sports 1998; 8: 203–207.Google Scholar
Galie N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006; 114: 48–54.Google Scholar
Konrad D, Oldner A, Rossi P, Wanecek M, Rudehill A, Weitzberg E. Differentiated and dose-related cardiovascular effects of a dual endothelin receptor antagonist in endotoxin shock. Crit Care Med 2004; 32: 1192–1199.Google Scholar
Konrad D, Oldner A, Wanecek M, et al. Positive inotropic and negative lusitropic effects of endothelin receptor agonism in vivo. Am J Physiol Heart Circ Physiol 2005; 289: H1702–H1709.Google Scholar
Wanecek M, Oldner A, Sundin P, Alving K, Weitzberg E, Rudehill A. Effects on haemodynamics by selective endothelin ET(B) receptor and combined endothelin ET(A)/ET(B) receptor antagonism during endotoxin shock. Eur J Pharmacol 1999; 386: 235–245.Google Scholar
Garofano RP, Barst RJ. Exercise testing in children with primary pulmonary hypertension. Pediatr Cardiol 1999; 20: 61–64.Google Scholar
Nagaya N, Uematsu M, Satoh T, et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999; 160: 487–492.Google Scholar