cardiovascularКардиоваскулярная терапия и профилактикаCardiovascular Therapy and Prevention1728-88002619-0125«SILICEA-POLIGRAF» LLCcardiovascular-1735Research ArticleОБЗОРЫREVIEWSСовременная медикаментозная терапия легочной артериальной гипертензииModern pharmaceutical treatment of pulmonary arterial hypertensionБатыралиевТ. А.BatyralievT. A.talantbekb@yahoo.comЭкинсиЭ.EkinsiE.talantbekb@yahoo.comНиязова-КарбенЗ. А.Niyazova-KarbenZ. A.talantbekb@yahoo.comПреображенскийД. В.PreobrazhenskyD. V.talantbekb@yahoo.comПатараяС. А.PatarayaS. A.talantbekb@yahoo.comМедицинский центр имени Сани Конукоглы и Университет. г. ГазиантепSani Konukogly Medical Center. GaziantepУчебно-научный медицинский центр Управления делами Президента Российской Федерации, г. МоскваEducational and Research Medical Center, RF President Administration. Moscow2008201220087894104Copyright © Батыралиев Т.А., Экинси Э., Ниязова-Карбен З.А., Преображенский Д.В., Патарая С.А., 20082008Батыралиев Т.А., Экинси Э., Ниязова-Карбен З.А., Преображенский Д.В., Патарая С.А.Batyraliev T.A., Ekinsi E., Niyazova-Karben Z.A., Preobrazhensky D.V., Pataraya S.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://cardiovascular.elpub.ru/jour/article/view/1735антагонисты кальцияпростаноидыэпопростенолтрепростенилилопростбозентансилденафилcalcium antagonistsprostanoidsepoprostenoltrepostinililoprostbosentansildenafilReferencesThe Task Force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. Eur Heart J 2004; 25(24): 2243-78.The Task Force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. Eur Heart J 2004; 25(24): 2243-78.Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. JACC 2004; 12: 40S-7.Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. JACC 2004; 12: 40S-7.Stewart S. Pulmonary arterial hypertension. London and New York 2005.Stewart S. Pulmonary arterial hypertension. London and New York 2005.Gabbay E, Reed A, Williams TJ. Asssessment and treatment of pulmonary arterial hypertension: an Australian perspective in 2006. Intern Med J 2007; 37: 38-48.Gabbay E, Reed A, Williams TJ. Asssessment and treatment of pulmonary arterial hypertension: an Australian perspective in 2006. Intern Med J 2007; 37: 38-48.Simonneau G, Gali N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. JACC 2004; 12: 5S-12.Simonneau G, Gali N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. JACC 2004; 12: 5S-12.Rich S. (ed.). Primary pulmonary hypertension. Executive summary from World symposium. Primary pulmonary hypertension 1998. Evian, France, WHO, 1998.Rich S. (ed.). Primary pulmonary hypertension. Executive summary from World symposium. Primary pulmonary hypertension 1998. Evian, France, WHO, 1998.D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with in primary pulmonary hypertension. Results from a National Prospective Registry. Ann Intern Med 1991; 115: 343-9.D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with in primary pulmonary hypertension. Results from a National Prospective Registry. Ann Intern Med 1991; 115: 343-9.McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension. ACCP evidence-based clinical practice guidelines. Chest 2004; 126: 78S-92.McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension. ACCP evidence-based clinical practice guidelines. Chest 2004; 126: 78S-92.Badesch DB, Ahman SH, Ahearn GS, et al. Medical therapy for pulmonary artery hypertension. ACCP evidence-based clinical practice guidelines. Chest 2004; 126: 35S-62.Badesch DB, Ahman SH, Ahearn GS, et al. Medical therapy for pulmonary artery hypertension. ACCP evidence-based clinical practice guidelines. Chest 2004; 126: 35S-62.Rich S, Seiditz M, Dodin E, et al. The short-term effects of digoxin in right ventricular dysfunction from pulmonary hypertension. Chest 1998; 114: 787-92.Rich S, Seiditz M, Dodin E, et al. The short-term effects of digoxin in right ventricular dysfunction from pulmonary hypertension. Chest 1998; 114: 787-92.Fuster V, Steele PM, Edwards WD, et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580-7.Fuster V, Steele PM, Edwards WD, et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580-7.Rich S, Kaufmann E, Levy PS. The effect of high dosed of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 76-81.Rich S, Kaufmann E, Levy PS. The effect of high dosed of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 76-81.Frank H, Mlczoch J, Huber K, et al. The effect of anticoagulant therapy in primary and anorectic drug-induced pulmonary hypertension. Chest 1997; 112: 714-21.Frank H, Mlczoch J, Huber K, et al. The effect of anticoagulant therapy in primary and anorectic drug-induced pulmonary hypertension. Chest 1997; 112: 714-21.Fortin TA, Tapson VF. Intravenous prostacyclin for pulmonary arterial hypertension. In: Pulmonary circulation. Diseases and their treatment. Peacock A.J., Rubin L. J. (eds). Second edition. London "Arnold" 2004; 255-67.Fortin TA, Tapson VF. Intravenous prostacyclin for pulmonary arterial hypertension. In: Pulmonary circulation. Diseases and their treatment. Peacock A.J., Rubin L. J. (eds). Second edition. London "Arnold" 2004; 255-67.Rubin LJ, Mendoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial therapy. Ann Intern Med 1990; 112: 485-91.Rubin LJ, Mendoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial therapy. Ann Intern Med 1990; 112: 485-91.Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334: 296-301.Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334: 296-301.Badesch D, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial therapy. Ann Intern Med 2000; 132: 425-34.Badesch D, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial therapy. Ann Intern Med 2000; 132: 425-34.McLaughlin VV, Genther DE, Panella MM, Rich S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998; 338: 273-7.McLaughlin VV, Genther DE, Panella MM, Rich S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998; 338: 273-7.Shapiro SM, Oudiz RJ, Cao T, et al. Primary pulmonary hypertension: Improved long-term effects and survival with continuous intravenous epoprostenol infusion. JACC 1997; 30: 343-9.Shapiro SM, Oudiz RJ, Cao T, et al. Primary pulmonary hypertension: Improved long-term effects and survival with continuous intravenous epoprostenol infusion. JACC 1997; 30: 343-9.McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: The impact of epoprostenol therapy. Circulation 2002; 106: 1477-82.McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: The impact of epoprostenol therapy. Circulation 2002; 106: 1477-82.Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: Prognostic factors and survival. JACC 2002; 40: 780-8.Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: Prognostic factors and survival. JACC 2002; 40: 780-8.McLaughlin VV. Oral and subcutaneous prostacyclin analogs. In: Pulmonary circulation. Diseases and their treatment. Peacock A. J., Rubin L. J. (eds). Second edition. London "Arnold" 2004; 278-82.McLaughlin VV. Oral and subcutaneous prostacyclin analogs. In: Pulmonary circulation. Diseases and their treatment. Peacock A. J., Rubin L. J. (eds). Second edition. London "Arnold" 2004; 278-82.Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of trepostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: A double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2002; 165: 800-4.Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of trepostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: A double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2002; 165: 800-4.Leuchte HH, Behr J. Iloprost for idiopathic pulmonary arterial hypertension. Expert Rev Cardiovasc Ther 2005; 3: 215-23.Leuchte HH, Behr J. Iloprost for idiopathic pulmonary arterial hypertension. Expert Rev Cardiovasc Ther 2005; 3: 215-23.Hsu HH, Rubin ZLJ. Iloprost inhalation solution for the treatment of pulmonary arterial hypertension. Expert Rev Cardiovasc Ther 2006; 6: 1921-30.Hsu HH, Rubin ZLJ. Iloprost inhalation solution for the treatment of pulmonary arterial hypertension. Expert Rev Cardiovasc Ther 2006; 6: 1921-30.Olschewski H, Seeger W. Inhaled iloprost. In: Pulmonary circulation. Diseases and their treatment. Peacock A. J., Rubin L. J. (eds). Second edition. London "Arnold" 2004; 268-77.Olschewski H, Seeger W. Inhaled iloprost. In: Pulmonary circulation. Diseases and their treatment. Peacock A. J., Rubin L. J. (eds). Second edition. London "Arnold" 2004; 268-77.Olschewski H, Simonneau G, Galie N, et al. for the Aerosolized Iloprost Randomized Study Group. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347: 322-9.Olschewski H, Simonneau G, Galie N, et al. for the Aerosolized Iloprost Randomized Study Group. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347: 322-9.Hoeper MM, Schwarze M, Ehlerding S, et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 2000; 342: 1866-70.Hoeper MM, Schwarze M, Ehlerding S, et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 2000; 342: 1866-70.Ono F, Nagaya N, Kyotani S, et al. Hemodynamic and hormonal effect of beraprost sodium, an oral active prostacyclin analogue, in patients with secondary precapillary pulmonary hypertension. Circulat J 2003; 67: 375-8.Ono F, Nagaya N, Kyotani S, et al. Hemodynamic and hormonal effect of beraprost sodium, an oral active prostacyclin analogue, in patients with secondary precapillary pulmonary hypertension. Circulat J 2003; 67: 375-8.Nagaya N, Uematsu M, Okano Y, et al. Effect of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: A randomized, double-blind, placebo-controlled trial. JACC 1999; 34: 1188-92.Nagaya N, Uematsu M, Okano Y, et al. Effect of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: A randomized, double-blind, placebo-controlled trial. JACC 1999; 34: 1188-92.Galie N, Humbert M, Vachiary J-L, et al., for the Arterial Pulmonary Hypertension And Beraprost European Trial (ALPHABET) Study Group. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. JACC 2002; 39: 1496-502.Galie N, Humbert M, Vachiary J-L, et al., for the Arterial Pulmonary Hypertension And Beraprost European Trial (ALPHABET) Study Group. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. JACC 2002; 39: 1496-502.Barst RJ, McGoon MD, McLaughlin V, et al., for the Beraprost Study Group. Beraprost therapy for pulmonary arterial hypertension. JACC 2003; 41: 2119-25.Barst RJ, McGoon MD, McLaughlin V, et al., for the Beraprost Study Group. Beraprost therapy for pulmonary arterial hypertension. JACC 2003; 41: 2119-25.Channick RN, Sitbon O, Barst RJ, et al. Endothelin receptor antagonists in pulmonary artery hypertension. JACC 2004; 43(Suppl.S): 62S-7.Channick RN, Sitbon O, Barst RJ, et al. Endothelin receptor antagonists in pulmonary artery hypertension. JACC 2004; 43(Suppl.S): 62S-7.Helmersen D, Channick RN, Rubin L. Endothelin receptor antagonists. In: Pulmonary circulation. Diseases and their treatment. Peacock A. J., Rubin L. J. (eds). Second edition. London "Arnold" 2004; 294-301.Helmersen D, Channick RN, Rubin L. Endothelin receptor antagonists. In: Pulmonary circulation. Diseases and their treatment. Peacock A. J., Rubin L. J. (eds). Second edition. London "Arnold" 2004; 294-301.Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary artery hypertension: A randomized placebo-controlled study. Lancet 2001; 358: 1119-23.Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary artery hypertension: A randomized placebo-controlled study. Lancet 2001; 358: 1119-23.Badesch DB, Bodin F, Channick RN, et al. Complete results of the first randomized, placebo-controlled study of bosentan, a dual endothelin receptor antagonist, in patients with pulmonary artery hypertension. CurrTher Res 2002; 63: 227-46.Badesch DB, Bodin F, Channick RN, et al. Complete results of the first randomized, placebo-controlled study of bosentan, a dual endothelin receptor antagonist, in patients with pulmonary artery hypertension. CurrTher Res 2002; 63: 227-46.Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary artery hypertension. N Engl J Med 2002; 346: 896-903.Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary artery hypertension. N Engl J Med 2002; 346: 896-903.McLaughlin VV, Sitbon O, BadeschDB, etal. Survival with first-line bosentan in patients with pulmonary artery hypertension. Eur Respir J 2005; 25: 244-9.McLaughlin VV, Sitbon O, BadeschDB, etal. Survival with first-line bosentan in patients with pulmonary artery hypertension. Eur Respir J 2005; 25: 244-9.Sitbon O, McLaughlin VV, Badesch DB, et al. Survival in patients with class III idiopathic pulmonary artery hypertension treated with first-line oral bosentan compared with historic cohort of patients started on intravenous epoprostenol. Thorax 2005; 60: 1025-30.Sitbon O, McLaughlin VV, Badesch DB, et al. Survival in patients with class III idiopathic pulmonary artery hypertension treated with first-line oral bosentan compared with historic cohort of patients started on intravenous epoprostenol. Thorax 2005; 60: 1025-30.Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary artery hypertension. Am J Respir Crit Care Med 2004; 169: 441-7.Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary artery hypertension. Am J Respir Crit Care Med 2004; 169: 441-7.Barst RJ, Langleben D, Langleben D, et al. Treatment of pulmonary artery hypertension with the selective endothelin-A receptor antagonist sitaxsentan. JACC 2006; 47: 2049-56.Barst RJ, Langleben D, Langleben D, et al. Treatment of pulmonary artery hypertension with the selective endothelin-A receptor antagonist sitaxsentan. JACC 2006; 47: 2049-56.Galia N, Badesch DB, Oudiz R, et al. Ambrisentan therapy for pulmonary artery hypertension. JACC 2005; 46: 529-35.Galia N, Badesch DB, Oudiz R, et al. Ambrisentan therapy for pulmonary artery hypertension. JACC 2005; 46: 529-35.Humbert M, Barst RJ, Robbins IM, et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Heart J 2004; 24: 353-9.Humbert M, Barst RJ, Robbins IM, et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Heart J 2004; 24: 353-9.Channick RN, Olschewski H, Seeger W, et al. Safety and effacy of inhaled treprostinil as add-on therapy to bosentan in pulmonary arterial hypertension. JACC 2006; 48: 1433-7.Channick RN, Olschewski H, Seeger W, et al. Safety and effacy of inhaled treprostinil as add-on therapy to bosentan in pulmonary arterial hypertension. JACC 2006; 48: 1433-7.Sastry BKS, Narasimhan C, Reddy NK, Raju BS. Clinical efficacy of sildenafil in primary pulmonary hypertension. JACC 2004; 43: 1149-53.Sastry BKS, Narasimhan C, Reddy NK, Raju BS. Clinical efficacy of sildenafil in primary pulmonary hypertension. JACC 2004; 43: 1149-53.Galia N, Ghofrani HA, Torbicki A. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148-57.Galia N, Ghofrani HA, Torbicki A. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148-57.Wilkins MR, Paul GA, Strange JW, et al. Sildenafil versus endothelin receptor antagonist for pulmonary hypertension (SERAPH) study. Am J Respir Crit Care Med 2005; 171: 1292-7.Wilkins MR, Paul GA, Strange JW, et al. Sildenafil versus endothelin receptor antagonist for pulmonary hypertension (SERAPH) study. Am J Respir Crit Care Med 2005; 171: 1292-7.
The authors declare that there are no conflicts of interest present.