Long-term efficacy of bosentan in treatment of pulmonary arterial hypertension in children

European Respiratory Journal

Volumn 38, Issue 1, 2011, Pages 70-77

  Hislop, A A ^a,b   Moledina, S ^a   Foster, H ^a   Schulze Neick, I ^a   Haworth, S G ^a,b  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

Bosentan; Idiopathic pulmonary arterial hypertension; Paediatric pulmonary arterial hypertension; Pulmonary hypertension in congenital heart disease

Indexed keywords

BOSENTAN; PROSTACYCLIN; SILDENAFIL;

ALANINE AMINOTRANSFERASE BLOOD LEVEL; ARTICLE; BODY HEIGHT; BODY WEIGHT; CHILD; CONGENITAL HEART DISEASE; CONTROLLED STUDY; DRUG DOSE REDUCTION; DRUG EFFICACY; DRUG SAFETY; DRUG SUBSTITUTION; DRUG WITHDRAWAL; EISENMENGER COMPLEX; FEMALE; HEMODYNAMICS; HUMAN; HYPOTENSION; INFANT; LIVER DYSFUNCTION; MAJOR CLINICAL STUDY; MALE; MONOTHERAPY; OBSERVATIONAL STUDY; PRIORITY JOURNAL; PULMONARY HYPERTENSION; RETROSPECTIVE STUDY; SCHOOL CHILD; SIDE EFFECT; SURVIVAL RATE; TREATMENT DURATION; UNITED KINGDOM; WALKING;

ADOLESCENT; ALGORITHMS; ANTIHYPERTENSIVE AGENTS; CHILD; CHILD, PRESCHOOL; DISEASE PROGRESSION; FEMALE; FOLLOW-UP STUDIES; HEART DEFECTS, CONGENITAL; HUMANS; HYPERTENSION, PULMONARY; MALE; PULMONARY MEDICINE; RETROSPECTIVE STUDIES; SULFONAMIDES; TREATMENT OUTCOME;

EID: 79960201162     PISSN: 09031936     EISSN: 13993003     Source Type: Journal    
DOI: 10.1183/09031936.00053510     Document Type: Article

References (26)

1. MacLean MR. Endothelin-1: a mediator of pulmonary hypertension? Pulm Pharmacol Ther 1998; 11: 125-132.
2. Allen SW, Chatfield BA, Koppenhafer SA, et al. Circulating immunoreactive endothelin-1 in children with pulmonary hypertension. Association with acute hypoxic pulmonary vasoreactivity. Am Rev Respir Dis 1993; 148: 519-522.
3. Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993; 328: 1732-1739. (Pubitemid 23170122)
4. Chen SJ, Chen YF, Meng QC, et al. Endothelin-receptor antagonist bosentan prevents and reverses hypoxic pulmonary hypertension in rats. J Appl Physiol 1995; 79: 2122-2131.
5. Holm P, Liska J, Clozel M, et al. The endothelin antagonist bosentan: hemodynamic effects during normoxia and hypoxic pulmonary hypertension in pigs. J Thorac Cardiovasc Surg 1996; 112: 890-897. (Pubitemid 26339260)
6. 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. (Pubitemid 32971843)
7. 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. (Pubitemid 36851359)
8. Provencher S, Sitbon O, Humbert M, et al. Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart J 2006; 27: 589-595.
9. Diller GP, Dimopoulos K, Kaya MG, et al. Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart 2007; 93: 974-976.
10. 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. (Pubitemid 36403597)
11. Rosenzweig EB, Ivy DD, Widlitz A, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005 16, 46: 697-704. (Pubitemid 41132700)
12. Maiya S, Hislop AA, Flynn Y, et al. Response to bosentan in children with pulmonary hypertension. Heart 2006; 92: 664-670.
13. Galie N, Torbicki A, Barst R, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J 2004; 25: 2243-2278.
14. Recommendations on the management of pulmonary hypertension in clinical practice. Heart 2001; 86: Suppl. 1, I1-I13.
15. Freeman JV, Cole TJ, Chinn S, et al. Cross sectional stature and weight reference curves for the UK, 1990. Arch Dis Child 1995; 73: 17-24.
16. Styles ME, Cole TJ, Dennis J, et al. New cross sectional stature, weight, and head circumference references for Down's syndrome in the UK and Republic of Ireland. Arch Dis Child 2002; 87: 104-108. (Pubitemid 34828011)
17. Geiger R, Strasak A, Treml B, et al. Six-minute walk test in children and adolescents. J Pediatr 2007; 150: 395-399.
18. Beghetti M, Hoeper MM, Kiely DG, et al. Safety experience with bosentan in 146 children 2-11 years old with pulmonary arterial hypertension: results from the European Postmarketing Surveillance program. Pediatr Res 2008; 64: 200-204.
19. Gilbert N, Luther YC, Miera O, et al. Initial experience with bosentan (Tracleer) as treatment for pulmonary arterial hypertension (PAH) due to congenital heart disease in infants and young children. Z Kardiol 2005; 94: 570-574. (Pubitemid 41276477)
20. Beghetti M, Haworth SG, Bonnet D, et al. Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with pulmonary arterial hypertension: the FUTURE-1 study. Br J Clin Pharmacol 2009; 68: 948-955.
21. McLaughlin VV. Survival in patients with pulmonary arterial hypertension treated with first-line bosentan. Eur J Clin Invest 2006; 36: Suppl. 3, 10-15. (Pubitemid 44179776)
22. Moledina S, Hislop AA, Foster H, et al. Childhood idiopathic pulmonary arterial hypertension - a national cohort study. Heart 2010; 96: 1401-1406.
23. Galie N, Rubin L, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 2008; 371: 2093-2100. (Pubitemid 351902178)
24. Gatzoulis MA, Beghetti M, Galie N, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol 2008 23, 127: 27-32. (Pubitemid 351707527)
25. van Loon RL, Hoendermis ES, Duffels MG, 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: 776-782.
26. Fasnacht MS, Tolsa JF, Beghetti M. The Swiss registry for pulmonary arterial hypertension: the paediatric experience. Swiss Med Wkly 2007; 137: 510-513. (Pubitemid 47441802)