systhiperСистемные гипертензииSystemic Hypertension2075-082X2542-2189LLC «ИнтерМедсервис»systhiper-464Research ArticleКАРДИОПУЛЬМОНОЛОГИЯСтратегия медикаментозного лечения легочной артериальной гипертензии в свете современных зарубежных рекомендацийStrategy of medical treatment of pulmonary arterial hypertension in the current international recommendationsМартынюкТ. В.MartyniukT. V.trukhiniv@mail.ruЧазоваИ. Е.ChazovaI. E.chazova@hotmail.comИнститут клинической кардиологии им. А.Л.Мясникова ФГБУ Российский кардиологический научно-производственный комплекс Минздрава РоссииРоссияA.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian FederationRussian Federation2016231220221324664Copyright © Мартынюк Т.В., Чазова И.Е., 20222022Мартынюк Т.В., Чазова И.Е.Martyniuk T.V., Chazova I.E.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.syst-hypertension.ru/jour/article/view/464

Существенный прогресс в лечении легочной артериальной гипертензии (ЛАГ), достигнутый за последние годы, связан с внедрением в клиническую практику целого ряда лекарственных препаратов патогенетического действия, способных вызывать вазодилатацию и обратное ремоделирование легочных сосудов. Недавно арсенал ЛАГ-специфической терапии пополнился новыми препаратами. Настоящий обзор создан в результате анализа современных американских рекомендаций CHEST и рекомендаций Европейского общества кардиологов и Европейского респираторного общества (ESC/ERS) для того, чтобы представить всем специалистам, занимающимся ведением ЛАГ, данные об основных подходах к фармакотерапии и оптимальном выборе фармакологических методов лечения.

Significant progress in the treatment of pulmonary arterial hypertension (PAH), in recent years, is associated with the introduction into clinical practice of a number of drugs pathogenetic action, can cause vasodilation and reverse remodeling of pulmonary vessels. Recently PAH-specific therapy was replenished with new drugs. This review is created as a result of the analysis of modern American recommendations CHEST and recommendations of the European society of cardiology and the European respiratory society (ESC/ERS) in order to provide all professionals involved in the maintenance of the PAH, data on the main approaches to the pharmacotherapy and the optimal choice of pharmacological treatment methods.

легочная гипертензияспецифическая терапиярекомендацииpulmonary hypertensionspecific therapyrecommendationsReferencesЧазова И.Е., Авдеев С.Н., Царева Н.А. и др. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевт. архив. 2014; 9: 4-23.Чазова И.Е., Авдеев С.Н., Царева Н.А. и др. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевт. архив. 2014; 9: 4-23.Channick R.N, Simonneau G, Sitbon O et al. Effects of the dual endothelin - receptor antagonist bosentan in patients with pulmonary hypertension: a randomisedplacebo - controlled study. Lancet 2001; 358 (9288): 1119-23.Channick R.N, Simonneau G, Sitbon O et al. Effects of the dual endothelin - receptor antagonist bosentan in patients with pulmonary hypertension: a randomisedplacebo - controlled study. Lancet 2001; 358 (9288): 1119-23.Barst R.J, Rubin L.J, Long W.A et al. Primary Pulmonary Hypertension Study Group. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334 (5): 296-301.Barst R.J, Rubin L.J, Long W.A et al. Primary Pulmonary Hypertension Study Group. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334 (5): 296-301.Galie N, Ghofrani H.A, Torbicki A et al. Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353 (20): 2148-57.Galie N, Ghofrani H.A, Torbicki A et al. Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353 (20): 2148-57.Ghofrani H.A, D’Armini A.M, Grimminger F et al. CHEST-1 Study Group. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369 (4): 319-29.Ghofrani H.A, D’Armini A.M, Grimminger F et al. CHEST-1 Study Group. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369 (4): 319-29.Taichman D.B, Ornelas J, Chung L et al. Pharmacologic therapy for Pulmonary Arterial Hypertension in Adults CHEST Guideline and Expert Panel Report. CHEST 2014; 146 (2): 449-75.Taichman D.B, Ornelas J, Chung L et al. Pharmacologic therapy for Pulmonary Arterial Hypertension in Adults CHEST Guideline and Expert Panel Report. CHEST 2014; 146 (2): 449-75.Galie N, Humbert M, Vachiery J.L et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Respir J 2015; 46 (4): 903-75.Galie N, Humbert M, Vachiery J.L et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Respir J 2015; 46 (4): 903-75.Mc Laughlin V.V, Gaine S.P, Howard L.S et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol 2013; 62 (Suppl. 25): D73-D81.Mc Laughlin V.V, Gaine S.P, Howard L.S et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol 2013; 62 (Suppl. 25): D73-D81.Lewis S.Z, Diekemper R, Ornelas J, Casey K.R. Methodologies for the development of CHEST guidelines and expert panel reports. Chest 2014; 146 (1): 182-92.Lewis S.Z, Diekemper R, Ornelas J, Casey K.R. Methodologies for the development of CHEST guidelines and expert panel reports. Chest 2014; 146 (1): 182-92.Sitbon O, Humbert M, Jais X et al. Long - term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111 (23): 3105-11.Sitbon O, Humbert M, Jais X et al. Long - term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111 (23): 3105-11.Humbert M, Barst R.J, Robbins I.M et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24: 353-9.Humbert M, Barst R.J, Robbins I.M et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24: 353-9.Sitbon O, Jaхђs X, Savale L et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691-7.Sitbon O, Jaхђs X, Savale L et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691-7.Galie` N, Barbera J.A, Frost A et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. New Engl J Med 2015; 379 (9): 834-44.Galie` N, Barbera J.A, Frost A et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. New Engl J Med 2015; 379 (9): 834-44.Fuster V, Steele P.M, Edwards W.D et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580-7.Fuster V, Steele P.M, Edwards W.D et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580-7.Herve P, Humbert M, Sitbon O et al. Pathobiology of pulmonary hypertension: the role of platelets and thrombosis. Clin Chest Med 2001; 22: 451-8.Herve P, Humbert M, Sitbon O et al. Pathobiology of pulmonary hypertension: the role of platelets and thrombosis. Clin Chest Med 2001; 22: 451-8.Hoeper M.M, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12: 1446-9.Hoeper M.M, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12: 1446-9.Sandoval J, Aguirre J.S, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682-7.Sandoval J, Aguirre J.S, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682-7.Rich S. Primary Pulmonary Hypertension: Executive Summary. Evian, France : World Health Organization, 1998.Rich S. Primary Pulmonary Hypertension: Executive Summary. Evian, France : World Health Organization, 1998.Galie N, Olschewski H, Oudiz R.J et al. Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies (ARIES) Group. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double - blind, placebo - controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008; 117 (23): 3010-9.Galie N, Olschewski H, Oudiz R.J et al. Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies (ARIES) Group. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double - blind, placebo - controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008; 117 (23): 3010-9.Galie N, Beghetti M, Gatzoulis M.A et al. Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double - blind, randomized, placebocontrolled study. Circulation 2006; 114 (1): 48-54.Galie N, Beghetti M, Gatzoulis M.A et al. Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double - blind, randomized, placebocontrolled study. Circulation 2006; 114 (1): 48-54.Galie N, Rubin L.j, Hoeper M et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double - blind, randomised controlled trial. Lancet 2008; 371 (9630): 2093-100.Galie N, Rubin L.j, Hoeper M et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double - blind, randomised controlled trial. Lancet 2008; 371 (9630): 2093-100.Pulido T, Adzerikho I, Channick R.N et al. SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369 (9): 809-18.Pulido T, Adzerikho I, Channick R.N et al. SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369 (9): 809-18.Simonneau G, Rubin L.J, Galie N et al. PACES Study Group.Addition of sildenafi to long - term intravenous epoprostenoltherapy in patients with pulmonary arterial hypertension: arandomized trial. Ann Intern Med 2008; 149 (8): 521-30.Simonneau G, Rubin L.J, Galie N et al. PACES Study Group.Addition of sildenafi to long - term intravenous epoprostenoltherapy in patients with pulmonary arterial hypertension: arandomized trial. Ann Intern Med 2008; 149 (8): 521-30.Galie N, Brundage B.H, Ghofrani H.A et al. Pulmonary Arterial Hypertension and Response to Tadalafi l (PHIRST) Study Group. Tadalafi l therapy for pulmonary arterial hypertension. Circulation 2009; 119 (22): 2894-903.Galie N, Brundage B.H, Ghofrani H.A et al. Pulmonary Arterial Hypertension and Response to Tadalafi l (PHIRST) Study Group. Tadalafi l therapy for pulmonary arterial hypertension. Circulation 2009; 119 (22): 2894-903.Galie N, Humbert M, Vachiery J.L et al. Effect of beraprostsodiim, an oral prostacyclin analogue, in pulmonary arterial hypertension: a randomized, double - blind placebo - controlled trial. J Am Coll Cardiol 2002; 39: 1496-502.Galie N, Humbert M, Vachiery J.L et al. Effect of beraprostsodiim, an oral prostacyclin analogue, in pulmonary arterial hypertension: a randomized, double - blind placebo - controlled trial. J Am Coll Cardiol 2002; 39: 1496-502.Olschevski H, Simonneau G, Galie N et al. Inhaled iloprost in severe pulmonary hypertension. N Engl J Med 2002; 347: 322-9.Olschevski H, Simonneau G, Galie N et al. Inhaled iloprost in severe pulmonary hypertension. N Engl J Med 2002; 347: 322-9.Mc Laughlin V.V, Oudiz R.J, Frost A et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension Am J Respir Crit Care Med 2006; 174: 1257-63.Mc Laughlin V.V, Oudiz R.J, Frost A et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension Am J Respir Crit Care Med 2006; 174: 1257-63.Lang I, Gomes-Sanches M, Kneussi M et al. Efficacy of long - term subcutaneous treprostinil sodium in pulmonary hypertension. Chest 2006; 129: 1636-43.Lang I, Gomes-Sanches M, Kneussi M et al. Efficacy of long - term subcutaneous treprostinil sodium in pulmonary hypertension. Chest 2006; 129: 1636-43.Badesch D.B, Tapson V.F, Mc Goon M.D et al. Continuous intravenous epoprostenol for pulmonary hypertension due to thescleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000; 132 (6): 425-34.Badesch D.B, Tapson V.F, Mc Goon M.D et al. Continuous intravenous epoprostenol for pulmonary hypertension due to thescleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000; 132 (6): 425-34.Galie` N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near - dead to multiple clinical trial meta - analyses. Eur Heart J 2010; 31: 2080-6.Galie` N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near - dead to multiple clinical trial meta - analyses. Eur Heart J 2010; 31: 2080-6.Rich S, Kaufmann E. High dose titration of calcium channel blockingagents for primary pulmonary hypertension: guidelines for short - term drug testing. J Am Coll Cardiol 1991; 18 (5): 1323-7.Rich S, Kaufmann E. High dose titration of calcium channel blockingagents for primary pulmonary hypertension: guidelines for short - term drug testing. J Am Coll Cardiol 1991; 18 (5): 1323-7.Ghofrani H-A et al. Riociguat for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med 2013; 369: 330-40. DOI: 10.1056/NEJMoa1209655 (+ supplementary materials; study protocol).Ghofrani H-A et al. Riociguat for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med 2013; 369: 330-40. DOI: 10.1056/NEJMoa1209655 (+ supplementary materials; study protocol).

The authors declare that there are no conflicts of interest present.