Survival in patients with pulmonary arterial hypertension treated with first-line bosentan
dc.contributor.author | McLaughlin, V. V. | en_US |
dc.date.accessioned | 2010-06-01T18:46:55Z | |
dc.date.available | 2010-06-01T18:46:55Z | |
dc.date.issued | 2006-09 | en_US |
dc.identifier.citation | McLaughlin, V. V. (2006). "Survival in patients with pulmonary arterial hypertension treated with first-line bosentan." European Journal of Clinical Investigation 36(s3): 10-15. <http://hdl.handle.net/2027.42/71978> | en_US |
dc.identifier.issn | 0014-2972 | en_US |
dc.identifier.issn | 1365-2362 | en_US |
dc.identifier.uri | https://hdl.handle.net/2027.42/71978 | |
dc.identifier.uri | http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=16919005&dopt=citation | en_US |
dc.description.abstract | Background Pulmonary arterial hypertension (PAH) is a devastating disease of the small pulmonary arteries and arterioles, characterized by intimal fibrosis, medial hypertrophy and plexiform lesions. When untreated both the idiopathic form (IPAH, formerly termed primary pulmonary hypertension, PPH) and PAH related to various other conditions such as scleroderma (SSc) often take a progressive course with high mortality. There is ongoing search for disease-specific treatments that are able to improve survival in these patients. The oral dual endothelin (ET A /ET B ) antagonist bosentan has been shown to improve exercise capacity, time to clinical worsening, haemodynamics and quality of life in short-term studies. Materials and methods To determine the long-term effects of bosentan on survival, patients from the two double-blind, randomized trials and their open-label extensions, treated with first-line bosentan, were followed for up to 3 years. Data on survival were collected between September 1999 (first patient included in the placebo-controlled trials) and December 2002. Vital status was verified in each patient. The survival cohorts of these patients were compared with either the predicted survival for each patient based on an equation from the National Institutes of Health (NIH) PPH registry or with historical controls. Results Observed survival up to 36 months was reported as a Kaplan-Meier estimate in three cohorts: (1) In 169 PPH patients treated with first-line bosentan, 1- and 2-year survival was 96% and 89%, respectively, vs. predicted untreated survival at 1 and 2 years of 69% and 57%, respectively; (2) in 50 patients with PAH associated with SSc (PAH-SSc), 1-, 2- and 3-year survival was 82%, 67% and 64%, respectively, vs. ∼45%, ∼35% and ∼28%, respectively, from registry data of untreated PAH-SSc patients; and (3) in 139 PPH patients in WHO functional class III, 1- and 2-year survival was 97% and 91%, respectively, vs. 91% and 84% in a historical cohort of 346 patients treated with epoprostenol in five major referral centres. Conclusions The present analyses suggest that first-line bosentan therapy, followed by the addition of other disease-specific therapies as required, improves survival in patients with advanced PAH. Eur J Clin Invest 2006; 36 (Suppl. 3): 10 –15 | en_US |
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dc.format.extent | 3109 bytes | |
dc.format.mimetype | application/pdf | |
dc.format.mimetype | text/plain | |
dc.publisher | Blackwell Publishing Ltd | en_US |
dc.rights | © 2006 The Author Journal compilation © 2006 Blackwell Publishing Ltd | en_US |
dc.subject.other | Bosentan | en_US |
dc.subject.other | Endothelin Receptor Antagonism | en_US |
dc.subject.other | Epoprostenol | en_US |
dc.subject.other | Hypertension | en_US |
dc.subject.other | Pulmonary | en_US |
dc.subject.other | Survival | en_US |
dc.title | Survival in patients with pulmonary arterial hypertension treated with first-line bosentan | en_US |
dc.type | Article | en_US |
dc.subject.hlbsecondlevel | Medicine (General) | en_US |
dc.subject.hlbtoplevel | Health Sciences | en_US |
dc.description.peerreviewed | Peer Reviewed | en_US |
dc.identifier.pmid | 16919005 | en_US |
dc.description.bitstreamurl | http://deepblue.lib.umich.edu/bitstream/2027.42/71978/1/j.1365-2362.2006.01688.x.pdf | |
dc.identifier.doi | 10.1111/j.1365-2362.2006.01688.x | en_US |
dc.identifier.source | European Journal of Clinical Investigation | en_US |
dc.identifier.citedreference | Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T 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 – 78. | en_US |
dc.identifier.citedreference | 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. | en_US |
dc.identifier.citedreference | Barst RJ, McGoon M, McLaughlin V, Tapson V, Rich S, Rubin L et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41: 2119 – 25. | en_US |
dc.identifier.citedreference | Opitz CF, Wensel R, Winkler J, Halank M, Bruch L, Kleber FX et al. Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J 2005; 26: 1895 – 902. | en_US |
dc.identifier.citedreference | Hoeper MM, Oudiz RJ, Peacock A, Tapson VF, Haworth SG, Frost AE et al. End points and clinical trial designs in pulmonary arterial hypertension: clinical and regulatory perspectives. J Am Coll Cardiol 2004; 43: 48S – 55S. | en_US |
dc.identifier.citedreference | Hoeper MM, Dinh-Xuan AT. Combination therapy for pulmonary arterial hypertension: still more questions than answers. Eur Respir J 2004; 24: 339 – 40. | en_US |
dc.identifier.citedreference | Galie N, Grigioni F, Bacchi-Reggiani L. Relation of endothelin-1 to survival in patients with primary pulmonary hypertension [abstract]. Eur J Clin Invest 1996; 26: A48. | en_US |
dc.identifier.citedreference | Rubens C, Ewert R, Halank M, Wensel R, Orzechowski HD, Schultheiss HP et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest 2001; 120: 1562 – 9. | en_US |
dc.identifier.citedreference | Jasmin JF, Lucas M, Cernacek P, Dupuis J. Effectiveness of a nonselective ET (A/B) and a selective ET (A) antagonist in rats with monocrotaline-induced pulmonary hypertension. Circulation 2001; 103: 314 – 8. | en_US |
dc.identifier.citedreference | Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001; 358: 1119 – 23. | en_US |
dc.identifier.citedreference | Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896 – 903. | en_US |
dc.identifier.citedreference | Galie N, Hinderliter AL, Torbicki A, Fourme T, Simonneau G, Pulido T et al. Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and doppler measures in patients with pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41: 1380 – 6. | en_US |
dc.identifier.citedreference | Sitbon O, Badesch DB, Channick RN, Frost A, Robbins IM, Simonneau G 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 – 54. | en_US |
dc.identifier.citedreference | McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galie N et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005; 25: 244 – 9. | en_US |
dc.identifier.citedreference | D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343 – 9. | en_US |
dc.identifier.citedreference | Sandoval J, Bauerle O, Palomar A, Gomez A, Martinez-Guerra ML, Beltran M et al. Survival in primary pulmonary hypertension. Validation of a prognostic equation. Circulation 1994; 89: 1733 – 44. | en_US |
dc.identifier.citedreference | Denton C, Humbert M, Rubin L, Coghlan J, Black C. Dual endothelin receptor antagonism in pulmonary arterial hypertension related to systemic sclerosis. Eur J Clin Invest 2005; 35 ( Suppl. 2 ). | en_US |
dc.identifier.citedreference | Koh E, Lee P, Gladman D, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Rheumatology 1996; 35: 989 – 93. | en_US |
dc.identifier.citedreference | Sitbon O, McLaughlin VV, Badesch DB, Barst RJ, Black C, Galie N et al. Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first-line oral bosentan compared with an historical cohort of patients started on i.v. epoprostenol. Thorax 2005; 60: 1025 – 30. | en_US |
dc.identifier.citedreference | Keogh A, Macdonald P, William T, McNeil K, Gabbay E, Proudman S et al. Bosentan for the treatment of pulmonary arterial hypertension – 6 month quality of life data [abstract]. J Heart Lung Transplant 2004; 23: S54. | en_US |
dc.identifier.citedreference | McLaughin VV, Hoeper MM. Pulmonary arterial hypertension: the race for the most effective treatment. Am J Respir Crit Care Med 2005; 171: 1199 – 201. | en_US |
dc.identifier.citedreference | Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 76 – 81. | en_US |
dc.identifier.citedreference | Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation 1999; 99: 1197 – 208. | en_US |
dc.identifier.citedreference | Yung D, Widlitz AC, Rosenzweig EB, Kerstein D, Maislin G, Barst RJ. Outcomes in Children With Idiopathic Pulmonary Arterial Hypertension. Circulation 2004; 110: 660 – 5. | en_US |
dc.identifier.citedreference | Sitbon O, Humbert M, Jais X, Ioos V, Hamid AM, Provencher S et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111: 3105 – 11. | en_US |
dc.identifier.citedreference | McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106: 1477 – 82. | en_US |
dc.identifier.citedreference | Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Herve P et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002; 40: 780 – 8. | en_US |
dc.identifier.citedreference | Shapiro SM, Oudiz RJ, Cao T, Romano MA, Beckmann XJ, Georgiou D et al. Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol 1997; 30: 343 – 9. | en_US |
dc.identifier.citedreference | Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 334: 296 – 302. | en_US |
dc.identifier.citedreference | Badesch DB, McLaughlin VV, Delcroix M, Vizza CD, Olschewski H, Sitbon O et al. Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43: 56S – 61S. | en_US |
dc.identifier.citedreference | Hoeper MM, Schwarze M, Ehlerding S, Adler-Schuermeyer A, Spiekerkoetter E, Niedermeyer J et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 2000; 342: 1866 – 70. | en_US |
dc.identifier.citedreference | Humbert M. Improving survival in pulmonary arterial hypertension. Eur Respir J 2005; 25: 218 – 20. | en_US |
dc.identifier.citedreference | Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123: 344 – 50. | en_US |
dc.owningcollname | Interdisciplinary and Peer-Reviewed |
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