Aguilar, R. V. and H. W. Farber. Epoprostenol (prostacyclin) therapy in HIV-associated pulmonary hypertension. Am J Respir Crit Care Med 162: 1846-50., 2000
Almagro, P., J. Julia, M. Sanjaume, et al. Pulmonary Capillary Hemangiomatosis Associated with Primary Pulmonary Hypertension: Report of 2 New Cases and Review of 35 Cases from the Literature. Medicine (Baltimore) 81: 417-24., 2002
Badesch, D. B., V. F. Tapson, M. D. McGoon, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 132: 425-34, 2000
Barst, R. J., M. McGoon, V. McLaughlin, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 41: 2119-25, 2003
Barst, R. J., L. J. Rubin, W. A. Long, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 334: 296-301, 1996
Barst, R. J., L. J. Rubin, M. D. McGoon, et al. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Ann Intern Med 121: 409-15, 1994
Bergstrom, S. and J. Sjovall. The isolation of prostaglandin. Acta Chem Scand 11: 1086-7, 1957
Bresser, P., P. F. Fedullo, W. R. Auger, et al. Continuous intravenous epoprostenol for chronic thromboembolic pulmonary hypertension. Eur Respir J 23: 595-600, 2004
Christman, B. W., C. D. McPherson, J. H. Newman, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. New Engl J Med 327: 70-5, 1992
Clapp, L. H., P. Finney, S. Turcato, et al. Differential effects of stable prostacyclin analogs on smooth muscle proliferation and cyclic AMP generation in human pulmonary artery (see comment). Am J Respir Cell Mol Biol 26: 194-201, 2002
D'Alonzo, G. E., R. J. Barst, S. M. Ayres, et al. Survival in patients with primary pulmonary hypertension. Ann Intern Med 115: 343-55, 1991
Davis, L. L., B. P. deBoisblanc, C. E. Glynn, et al. Effect of prostacyclin on microvascular pressures in a patient with pulmonary veno-occlusive disease. Chest 108: 1754-6, 1754
de la Mata, J., M. A. Gomez-Sanchez, M. Aranzana, et al. Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases. Arthritis Rheum 37: 1528-33, 1994
Dusting, G. J., S. Moncada and J. R. Vane. Prostaglandins, their intermediates and precursors: cardiovascular actions and regulatory roles in normal and abnormal circulatory systems. Prog Cardiovasc Dis 21: 405-30, 1979
Eronen, M., M. Pohjavuori, S. Andersson, et al. Prostacyclin treatment for persistent pulmonary hypertension of the newborn. Pediatr Cardiol 18: 3-7., 1997
Farber, H. W., K. K. Graven, G. Kokolski, et al. Pulmonary edema during acute infusion of epoprostenol in a patient with pulmonary hypertension and limited scleroderma. J Rheumatol 26: 1195-6, 1999
Galie, N., M. Humbert, J. L. Vachiery, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 39: 1496-502., 2002
Galie, N., W. Seeger, R. Naeije, et al. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. Journal of the American College of Cardiology 43: 16, 2004
Geraci, M. W., B. Gao, D. C. Shepherd, et al. Pulmonary prostacyclin synthase overexpression in transgenic mice protects against development of hypoxic pulmonary hypertension. J Clin Invest 103: 1509-15., 1999
Ghofrani, H. A., G. Friese, T. Discher, et al. Inhaled iloprost is a potent acute pulmonary vasodilator in HIV-related severe pulmonary hypertension. Eur Respir J 23: 321-6, 2004
Gomez-Sanchez, M. A., C. S. de la Calzada, C. Gomez Pajuelo, et al. Different hemodynamic responses between acute and chronic infusion of iloprost (prostacyclin-stable analogue) in severe pulmonary hypertension. Am Rev Respir Dis 144: 1404-5, 1991
Higenbottam, T., A. Y. Butt, A. McMahon, et al. Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart 80: 151-5, 1998
Higenbottam, T., D. Wheeldon, F. Wells, et al. Long-term treatment of primary pulmonary hypertension with continuous intravenous epoprostenol (prostacyclin). Lancet 1: 1046-7, 1984
Higenbottam, T. W., A. Y. Butt, A. T. Dinh-Xaun, et al. Treatment of pulmonary hypertension with the continuous infusion of a prostacyclin analogue, iloprost. Heart 79: 175-9., 1998
Hoeper, M. M., M. Schwarze, S. Ehlerding, et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 342: 1866-70, 2000
Hoeper, M. M., E. Spiekerkoetter, V. Westerkamp, et al. Intravenous iloprost for treatment failure of aerosolised iloprost in pulmonary arterial hypertension. Eur Respir J 20: 339-43., 2002
Humbert, M., S. Maitre, F. Capron, et al. Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med 157: 1681-5, 1998
Humbert, M., O. Sanchez, M. Fartoukh, et al. Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study. Eur Respir J 13: 1351-6, 1999
Ivy, D. D., A. Doran, L. Claussen, et al. Weaning and discontinuation of epoprostenol in children with idiopathic pulmonary arterial hypertension receiving concomitant bosentan. Am J Cardiol 93: 943-6, 2004
Jones, D. K., T. W. Higenbottam and J. Wallwork. Treatment of primary pulmonary hypertension intravenous epoprostenol (prostacyclin). Br Heart J 57: 270-8, 1987
Kim, N. H., R. N. Channick and L. J. Rubin. Successful withdrawal of long-term epoprostenol therapy for pulmonary arterial hypertension. Chest 124: 1612-5, 2003
Krause, W. and T. Krais. Pharmacokinetics and pharmacodynamics of the prostacyclin analogue iloprost in man. Eur J Clin Pharmacol 30: 61-8., 1986
Krowka, M. J., R. P. Frantz, M. D. McGoon, et al. Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): A study of 15 patients with moderate to severe portopulmonary hypertension. Hepatology 30: 641-8., 1999
Kuhn, K. P., D. W. Byrne, P. G. Arbogast, et al. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med 167: 580-6, 2003
Kuhn, K. P., N. E. Wickersham, I. M. Robbins, et al. Acute effects of sildenafil in patients with primary pulmonary hypertension receiving epoprostenol. Exp Lung Res 30: 135-45, 2004
Kuo, P. C., L. B. Johnson, J. S. Plotkin, et al. Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension. Transplantation 63: 604-6., 1997
Langleben, D., B. W. Christman, R. J. Barst, et al. Effects of the thromboxane synthetase inhibitor and receptor antagonist terbogrel in patients with primary pulmonary hypertension. Am Heart J 143: e4, 2002
Lock, J. E., P. M. Olley, F. Coceani, et al. Use of prostacyclin in persistent fetal circulation. Lancet 1: 23, 1979
Long, W. A. and L. J. Rubin. Prostacyclin and PGE1 treatment of pulmonary hypertension. Am Rev Respir Dis 136: 773-6, 1987
Machherndl, S., M. Kneussl, H. Baumgartner, et al. Long-term treatment of pulmonary hypertension with aerosolized iloprost. Eur Respir J 17: 8-13, 2001
McLaughlin, V. V., D. E. Genthner, M. M. Panella, et al. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. New Engl J Med 338: 273-7, 1998
McLaughlin, V. V., A. Shillington and S. Rich. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 106: 1477-82., 2002
Moncada, S., R. Gryglewski, S. Bunting, et al. An enzyme isolated from arteries transforms prostaglandin endoperoxides to an unstable substance that inhibits platelet aggregation. Nature 263: 663-5, 1976
Moncada, S. and J. R. Vane. Arachidonic acid metabolites and the interactions between platelets and blood-vessel walls. New England Journal of Medicine 300: 1142-7, 1979
Morassut, P. A., V. M. Walley and C. D. Smith. Pulmonary veno-occlusive disease and the CREST variant of scleroderma. Can J Cardiol 8: 1055-8, 1992
Naeye, R. L. Pulmonary vascular lesions in systemic sclerosis. Dis Chest 44: 374-80, 1963
Nagaya, N., N. Sasaki, M. Ando, et al. Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Chest 123: 338-43., 2003
Nunes, H., M. Humbert, O. Sitbon, et al. Prognostic factors for survival in human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 167: 1433-9., 2003
Olschewski, H., H. A. Ghofrani, T. Schmehl, et al. Inhaled iloprost to treat severe pulmonary hypertension. An uncontrolled trial. German PPH Study Group. Ann Intern Med 132: 435-43., 2000
Olschewski, H., H. A. Ghofrani, D. Walmrath, et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med 160: 600-7., 1999
Olschewski, H., G. Simonneau, N. Galie, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 347: 322-9., 2002
Olschewski, H., D. Walmrath, R. Schermuly, et al. Aerosolized prostacyclin and iloprost in severe pulmonary hypertension. Ann Intern Med 124: 820-4, 1996
Palmer, S. M., L. J. Robinson, A. Wang, et al. Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest 113: 237-40, 1998
Petitpretz, P., F. Brenot, R. Azarian, et al. Pulmonary hypertension in patients with human immunodeficiency virus infection. Comparison with primary pulmonary hypertension. Circulation 89: 2722-7, 1994
Rich, S. and V. V. McLaughlin. The effects of chronic prostacyclin therapy on cardiac output and symptoms in primary pulmonary hypertension (see comment). J Am Coll Cardiol 34: 1184-7, 1999
Robbins, I. M., S. P. Gaine, R. Schilz, et al. Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest 117: 14-8, 2000
Rosenzweig, E. B., D. Kerstein and R. J. Barst. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 99: 1858-65., 1999
Rubin, L. J., J. Mendoza, M. Hood, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 112: 485-91, 1990
Shapiro, S. M., R. J. Oudiz, T. Cao, et al. Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol 30: 343-9, 1997
Simonneau, G., R. J. Barst, N. Galie, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 165: 800-4., 2002
Sitbon, O., M. Humbert, H. Nunes, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 40: 780-8., 2002
Stiebellehner, L., V. Petkov, K. Vonbank, et al. Long-term treatment with oral sildenafil in addition to continuous IV epoprostenol in patients with pulmonary arterial hypertension. Chest 123: 1293-5., 2003
Strange, C., M. Bolster, J. Mazur, et al. Hemodynamic effects of epoprostenol in patients with systemic sclerosis and pulmonary hypertension. Chest 118: 1077-82, 2000
Stricker, H., G. Domenighetti, G. Fiori, et al. Sustained improvement of performance and haemodynamics with long-term aerosolised prostacyclin therapy in severe pulmonary hypertension. Schweiz Med Wochenschr 129: 923-7., 1999
Tuder, R. M., C. D. Cool, M. W. Geraci, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 159: 1925-32., 1999
Vachiery, J. L., N. Hill, D. Zwicke, et al. Transitioning from i.v. epoprostenol to subcutaneous treprostinil in pulmonary arterial hypertension. Chest 121: 1561-5, 1561
Von Euler, U. S. On the specific vasodilating and plain muscle stimulating substance from accessory genital glands in man and certain animals (prostaglandin and vesiglandin). J Physiol (Lond) 88: 213-34, 1936
Walmrath, D., R. Schermuly, J. Pilch, et al. Effects of inhaled versus intravenous vasodilators in experimental pulmonary hypertension. Eur Respir J 10: 1084-92, 1997
Walmrath, D., T. Schneider, J. Pilch, et al. Aerosolised prostacyclin in adult respiratory distress syndrome. Lancet 342: 961-2, 1993
Watkins, W. D., M. B. Peterson, R. K. Crone, et al. Prostacyclin and prostaglandin E1 for severe idiopathic pulmonary artery hypertension. Lancet 1: 17, 1980
Wax, D., R. Garofano and R. J. Barst. Effects of long-term infusion of prostacyclin on exercise performance in patients with primary pulmonary hypertension. Chest 116: 914-20, 1999
Wilkens, H., A. Guth, J. Konig, et al. Effect of inhaled iloprost plus oral sildenafil in patients with primary pulmonary hypertension. Circulation 104: 1218-22., 2001
Yousem, S. A. The pulmonary pathologic manifestations of the CREST syndrome. Hum Pathol 21: 467-74, 1990