Circulation. 2002;106:1477-1482
Published online before print August 26, 2002, doi: 10.1161/01.CIR.0000029100.82385.58
Published online before print August 26, 2002, doi: 10.1161/01.CIR.0000029100.82385.58
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(Circulation. 2002;106:1477.)
© 2002 American Heart Association, Inc.
Clinical Investigation and Reports |
Survival in Primary Pulmonary Hypertension
The Impact of Epoprostenol Therapy
From Rush-Presbyterian-St Luke’s Medical Center, Department of Medicine, Section of Cardiology, Chicago, Ill (V.V.M., S.R.), and EPI-Q Inc, Oakbrook Terrace, Ill (A.S.).
Correspondence to Vallerie V. McLaughlin, MD, Rush-Presbyterian-St Luke’s Medical Center, 1725 W. Harrison Street, Suite 020, Chicago, IL 60612. E-mail vmclaugh{at}rush.edu
Background— Primary pulmonary hypertension (PPH) is a severe and progressive disease. Without treatment, the median survival is 2.8 years, with survival rates of 68%, 48%, and 34% at 1, 3, and 5 years, respectively. Intravenous epoprostenol was the first Food and Drug Administration–approved therapy for PPH. The long-term impact that epoprostenol has made on PPH remains to be defined.
Methods and Results— One hundred sixty-two consecutive patients diagnosed with PPH and treated with epoprostenol were followed for a mean of 36.3 months (median, 31 months). Data including functional class, exercise tolerance, and hemodynamics were recorded in a customized database. Vital status was verified in each patient. Observed survival with epoprostenol therapy at 1, 2, and 3 years was 87.8%, 76.3%, and 62.8% and was significantly greater than the expected survival of 58.9%, 46.3%, and 35.4% based on historical data. Baseline predictors of survival included exercise tolerance, functional class, right atrial pressure, and vasodilator response to adenosine. Predictors of survival after the first year of therapy included functional class and improvement in exercise tolerance, cardiac index, and mean pulmonary artery pressure.
Conclusions— Intravenous epoprostenol improves long-term survival in PPH.
Key Words: pulmonary heart disease • prostaglandins • survival
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M. M. Hoeper, I. Markevych, E. Spiekerkoetter, T. Welte, and J. Niedermeyer Goal-oriented treatment and combination therapy for pulmonary arterial hypertension Eur. Respir. J., November 1, 2005; 26(5): 858 - 863. [Abstract] [Full Text] [PDF]
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D. Montani, L. Achouh, A. G. Marcelin, J-P. Viard, O. Hermine, D. Canioni, O. Sitbon, G. Simonneau, and M. Humbert Reversibility of pulmonary arterial hypertension in HIV/HHV8-associated Castleman's disease Eur. Respir. J., November 1, 2005; 26(5): 969 - 972. [Abstract] [Full Text] [PDF]
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A. Ogawa, K. Nakamura, H. Matsubara, H. Fujio, T. Ikeda, K. Kobayashi, I. Miyazaki, M. Asanuma, K. Miyaji, D. Miura, et al. Prednisolone Inhibits Proliferation of Cultured Pulmonary Artery Smooth Muscle Cells of Patients With Idiopathic Pulmonary Arterial Hypertension Circulation, September 20, 2005; 112(12): 1806 - 1812. [Abstract] [Full Text] [PDF]
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I. Adatia Improving the Outcome of Childhood Pulmonary Arterial Hypertension: The Effect of Bosentan in the Setting of a Dedicated Pulmonary Hypertension Clinic J. Am. Coll. Cardiol., August 16, 2005; 46(4): 705 - 706. [Full Text] [PDF]
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L. J. Rubin and D. B. Badesch Evaluation and Management of the Patient with Pulmonary Arterial Hypertension Ann Intern Med, August 16, 2005; 143(4): 282 - 292. [Abstract] [Full Text] [PDF]
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M. Oikawa, Y. Kagaya, H. Otani, M. Sakuma, J. Demachi, J. Suzuki, T. Takahashi, J. Nawata, T. Ido, J. Watanabe, et al. Increased [18F]Fluorodeoxyglucose Accumulation in Right Ventricular Free Wall in Patients With Pulmonary Hypertension and the Effect of Epoprostenol J. Am. Coll. Cardiol., June 7, 2005; 45(11): 1849 - 1855. [Abstract] [Full Text] [PDF]
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M. R. Wilkins, G. A. Paul, J. W. Strange, N. Tunariu, W. Gin-Sing, W. A. Banya, M. A. Westwood, A. Stefanidis, L. L. Ng, D. J. Pennell, et al. Sildenafil versus Endothelin Receptor Antagonist for Pulmonary Hypertension (SERAPH) Study Am. J. Respir. Crit. Care Med., June 1, 2005; 171(11): 1292 - 1297. [Abstract] [Full Text] [PDF]
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A. Vonk-Noordegraaf, S. A. van Wolferen, J. T. Marcus, A. Boonstra, P. E. Postmus, J. W. L. Peeters, and A. J. Peacock Noninvasive assessment and monitoring of the pulmonary circulation Eur. Respir. J., April 1, 2005; 25(4): 758 - 766. [Abstract] [Full Text] [PDF]
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S. D. Nathan Lung Transplantation: Disease-Specific Considerations for Referral Chest, March 1, 2005; 127(3): 1006 - 1016. [Abstract] [Full Text] [PDF]
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M. Humbert Improving survival in pulmonary arterial hypertension Eur. Respir. J., February 1, 2005; 25(2): 218 - 220. [Full Text] [PDF]
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V. V. McLaughlin, O. Sitbon, D. B. Badesch, R. J. Barst, C. Black, N. Galie, M. Rainisio, G. Simonneau, and L. J. Rubin Survival with first-line bosentan in patients with primary pulmonary hypertension Eur. Respir. J., February 1, 2005; 25(2): 244 - 249. [Abstract] [Full Text] [PDF]
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Task Force members, N. Galie, A. Torbicki, R. Barst, P. Dartevelle, S. Haworth, T. Higenbottam, H. Olschewski, A. Peacock, G. Pietra, 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., December 2, 2004; 25(24): 2243 - 2278. [Full Text] [PDF]
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M.M. Hoeper, C. Faulenbach, H. Golpon, J. Winkler, T. Welte, and J. Niedermeyer Combination therapy with bosentan and sildenafil in idiopathic pulmonary arterial hypertension Eur. Respir. J., December 1, 2004; 24(6): 1007 - 1010. [Abstract] [Full Text] [PDF]
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D. Langleben, A. M. Hirsch, E. Shalit, L. Lesenko, and R. J. Barst Sustained Symptomatic, Functional, and Hemodynamic Benefit With the Selective Endothelin-A Receptor Antagonist, Sitaxsentan, in Patients With Pulmonary Arterial Hypertension: A 1-Year Follow-up Study Chest, October 1, 2004; 126(4): 1377 - 1381. [Abstract] [Full Text] [PDF]
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M.M. Hoeper and A.T. Dinh-Xuan Combination therapy for pulmonary arterial hypertension: still more questions than answers Eur. Respir. J., September 1, 2004; 24(3): 339 - 340. [Full Text] [PDF]
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M. Humbert, R.J. Barst, I.M. Robbins, R.N. Channick, N. Galie, A. Boonstra, L.J. Rubin, E.M. Horn, A. Manes, and G. Simonneau Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2 Eur. Respir. J., September 1, 2004; 24(3): 353 - 359. [Abstract] [Full Text] [PDF]
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D. Yung, A. C. Widlitz, E. B. Rosenzweig, D. Kerstein, G. Maislin, and R. J. Barst Outcomes in Children With Idiopathic Pulmonary Arterial Hypertension Circulation, August 10, 2004; 110(6): 660 - 665. [Abstract] [Full Text] [PDF]
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