Bosentan therapy in patients with Eisenmenger syndrome: A multicenter, double-blind, randomized, placebo-controlled study

Circulation

Volumn 114, Issue 1, 2006, Pages 48-54

  Galiè, Nazzareno ^a   Beghetti, Maurice ^b   Gatzoulis, Michael A ^c,d   Granton, John ^e   Berger, Rolf M F ^f   Lauer, Andrea ^g   Chiossi, Eleonora ^g   Landzberg, Michael ^h  

^a UNIVERSITY OF BOLOGNA   (Italy)

^b Hopital des Enfants Hopitaux universitaires de Geneve   (Switzerland)

^c ROYAL BROMPTON HOSPITAL   (United Kingdom)

^d NATIONAL HEART AND LUNG INSTITUTE   (United Kingdom)

^e TORONTO GENERAL HOSPITAL   (Canada)

^f UNIVERSITY MEDICAL CENTER GRONINGEN   (Netherlands)

^g ACTELION PHARMACEUTICALS LTD   (Switzerland)

^h BOSTON CHILDREN S HOSPITAL   (United States)

Author keywords

Endothelin; Heart septal defects; Hypertension; Pulmonary

Indexed keywords

BOSENTAN; ENDOTHELIN RECEPTOR ANTAGONIST; PLACEBO; ANTIHYPERTENSIVE AGENT; ENDOTHELIN RECEPTOR; OXYGEN; SULFONAMIDE;

ADULT; ANGINA PECTORIS; ARTERIAL OXYGEN SATURATION; ARTICLE; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; DISEASE SEVERITY; DIZZINESS; DOUBLE BLIND PROCEDURE; DRUG EFFECT; DRUG INDUCED HEADACHE; DRUG SAFETY; EISENMENGER COMPLEX; EXERCISE; FATIGUE; FEMALE; HEART CATHETERIZATION; HEART HEMODYNAMICS; HEART PALPITATION; HEMODYNAMIC PARAMETERS; HUMAN; HYPOXEMIA; LUNG ARTERY PRESSURE; LUNG VASCULAR RESISTANCE; MALE; MULTICENTER STUDY; PERIPHERAL EDEMA; PRIORITY JOURNAL; PULMONARY HYPERTENSION; PULSE OXIMETRY; RANDOMIZED CONTROLLED TRIAL; THORAX PAIN; WORLD HEALTH ORGANIZATION; DRUG ANTAGONISM; EXERCISE TOLERANCE; HEMODYNAMICS; MIDDLE AGED; PATHOPHYSIOLOGY; TREATMENT OUTCOME;

ADULT; ANTIHYPERTENSIVE AGENTS; DOUBLE-BLIND METHOD; EISENMENGER COMPLEX; EXERCISE TOLERANCE; FEMALE; HEMODYNAMIC PROCESSES; HUMANS; MALE; MIDDLE AGED; OXYGEN; RECEPTORS, ENDOTHELIN; SULFONAMIDES; TREATMENT OUTCOME;

EID: 33746206340     PISSN: 00097322     EISSN: None     Source Type: Journal    
DOI: 10.1161/CIRCULATIONAHA.106.630715     Document Type: Article

References (22)

1. Berman EB, Barst RJ. Eisenmenger's syndrome: current management. Prog Cardiovasc Dis. 2002;45:129-138.
2. Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F, Hoffman A Sorenson K, Kaemmer H, Thilen U, Bink-Boelkens M, Iserin L, Daliento L, Silove E, Redington A, Vouhe P. Management of grown up congenital heart disease. Eur Heart J. 2003;24:1035-1084.
3. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med. 1998;128:745-755.
4. Pietra GG, Capron F, Stewart S, Leone O, Humber DM, Reid L, Grimminger F, Robbins I, Tuder RM. Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol. 2004;43(suppl 1):S25-S32.
5. Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, Stone S. Eisenmenger syndrome: factors relating to deterioration and death. Eur Heart J. 1998;19:1845-1855.
6. Diller GP, Dimopoulos K, Okonko D, Li W, Babu-Narayan SV, Broberg CS, Johansson B, Bouzas B, Mullen MJ, Poole-Wilson PA, Francio DP, Gatzoulis MA. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828-835.
7. Oya H, Nagaya N, Uematsu M, Satoh T, Sakamaki F, Kyotani S, Sato N, Nakanishi N, Miyatake K. Poor prognosis and related factors in adults with Eisenmenger syndrome. Am Heart J. 2002;143:739-744.
8. Galie N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc Res. 2004;61:227-237.
9. Humbert M, Morrell N, Archer S, Stenmark KR, McLean M, Lang I, Christman B, Weir EK, Eickleberg O, Voelkel N, Rabinovitch M. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43(suppl 1):S13-S24.
10. Cacoub P, Dorent R, Maistre G, Nataf P, Carayon A, Piette C, Godeau P, Gandjbakhch I. Endothelin-1 in primary pulmonary hypertension and the Eisenmenger syndrome. Am J Cardiol. 1993;71:448-450.
11. Giaid A, Yanagisawa M, Langleben D, Michel RP, Levy R, Shennib H, Rimura S, Masaki T, Duguid WP, Stewart DJ. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med. 1993;328:1732-1739.
12. Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF, Badesch DB, Roux S, Rainisio M, Bodin F, Rubin LJ. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet. 2001;358:1119-1123.
13. Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, Pulido T, Frost A, Roux S, Leconte I, Landzberg M, Simonneau G. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346:896-903.
14. 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.
15. Gatzoulis MA, Rogers P, Li W, Harries C, Cramer D, Ward S, Mikhail G, Gibbs JS. Safety and tolerability of bosentan in adults with Eisenmenger physiology. Int J Cardiol. 2005;98:147-151.
16. 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 .
17. Schulze-Neick I, Gilbert N, Ewert R, Witt C, Gruenig E, Enke B, Borst MM, Lange PE, Hoeper MM. Adult patients with congenital heart disease and pulmonary arterial hypertension: first open prospective multicenter study of bosentan therapy. Am Heart J. 2005;150:716.e7-716.e12.
18. Galie N. Do we need controlled clinical trials in pulmonary arterial hypertension? Eur Respir J. 2001;17:1-3.
19. Enright PL, Sherril DL. Reference equations for the six-minute walk in healthy adults. Am J Respir Crit Care Med. 1998;158:1384-1387.
20. Miyamoto S, Nagaya N, Satoh T, Kyotani S, Sakamaki F, Fujita M, Nakanishi N, Miyatake K. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension: comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2000;161(pt 1):487-492.
21. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004;351:1425-1436.
22. Hodges LD, Brodie DA, Bromley PD. Validity and reliability of selected commercially available metabolic analyzer systems. Scand J Med Sci Sports. 2005;15:271-279.