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Стратегия медикаментозного лечения легочной артериальной гипертензии в свете современных зарубежных рекомендаций
Стратегия медикаментозного лечения легочной артериальной гипертензии в свете современных зарубежных рекомендаций
Мартынюк Т.В., Чазова И.Е. Стратегия медикаментозного лечения легочной артериальной гипертензии в свете современных зарубежных рекомендаций. Системные гипертензии. 2016; 13 (2): 46–64.
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Аннотация
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.
Ключевые слова: легочная гипертензия, специфическая терапия, рекомендации.
Key words: pulmonary hypertension, specific therapy, recommendations.
Ключевые слова: легочная гипертензия, специфическая терапия, рекомендации.
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Key words: pulmonary hypertension, specific therapy, recommendations.
Полный текст
Список литературы
1. 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. / Chazova I.E., Avdeev S.N., Tsareva N.A. i dr. Klinicheskie rekomendatsii po diagnostike i lecheniiu legochnoi gipertonii. Terapevt. arkhiv. 2014; 9: 4–23. [in Russian]
2. Channick RN, 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.
3. Barst RJ, Rubin LJ, Long WA 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.
4. Galie N, Ghofrani HA, 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.
5. Ghofrani HA, D’Armini AM, 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.
6. Taichman DB, 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.
7. Galie N, Humbert M, Vachiery JL 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.
8. McLaughlin VV, Gaine SP, Howard LS et al. Treatment goals of pulmonary hypertension. J Am CollCardiol 2013; 62 (Suppl. 25): D73–D81.
9. Lewis SZ, Diekemper R, Ornelas J, Casey KR. Methodologies for the development of CHEST guidelines and expert panel reports. Chest 2014; 146 (1): 182–92.
10. 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.
11. Humbert M, Barst RJ, Robbins IM et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24: 353–9.
12. Sitbon O, Jaı¨s X, Savale L et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691–7.
13. Galie` N, Barbera JA, Frost A et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. New Engl J Med 2015; 379 (9): 834–44.
14. Fuster V, Steele PM, Edwards WD et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580–7.
15. 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.
16. Hoeper MM, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12: 1446–9.
17. Sandoval J, Aguirre JS, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682–7.
18. Rich S. Primary Pulmonary Hypertension: Executive Summary. Evian, France : World Health Organization, 1998.
19. Galie N, Olschewski H, Oudiz RJ 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.
20. Galie N, Beghetti M, Gatzoulis MA 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.
21. Galie N, Rubin Lj, 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.
22. Pulido T, Adzerikho I, Channick RN et al. SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369 (9): 809–18.
23. Simonneau G, Rubin LJ, 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.
24. Galie N, Brundage BH, Ghofrani HA 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.
25. Galie N, Humbert M, Vachiery JL 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.
26. Olschevski H, Simonneau G, Galie N et al. Inhaled iloprost in severe pulmonary hypertension. N Engl J Med 2002; 347: 322–9.
27. McLaughlin VV, Oudiz RJ, 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.
28. Lang I, Gomes-Sanches M, Kneussi M et al. Efficacy of long-term subcutaneous treprostinil sodium in pulmonary hypertension. Chest 2006; 129: 1636–43.
29. Badesch DB, Tapson VF, McGoon MD 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.
30. 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.
31. 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.
32. 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).
2. Channick RN, 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.
3. Barst RJ, Rubin LJ, Long WA 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.
4. Galie N, Ghofrani HA, 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.
5. Ghofrani HA, D’Armini AM, 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.
6. Taichman DB, 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.
7. Galie N, Humbert M, Vachiery JL 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.
8. McLaughlin VV, Gaine SP, Howard LS et al. Treatment goals of pulmonary hypertension. J Am CollCardiol 2013; 62 (Suppl. 25): D73–D81.
9. Lewis SZ, Diekemper R, Ornelas J, Casey KR. Methodologies for the development of CHEST guidelines and expert panel reports. Chest 2014; 146 (1): 182–92.
10. 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.
11. Humbert M, Barst RJ, Robbins IM et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24: 353–9.
12. Sitbon O, Jaı¨s X, Savale L et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691–7.
13. Galie` N, Barbera JA, Frost A et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. New Engl J Med 2015; 379 (9): 834–44.
14. Fuster V, Steele PM, Edwards WD et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580–7.
15. 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.
16. Hoeper MM, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12: 1446–9.
17. Sandoval J, Aguirre JS, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682–7.
18. Rich S. Primary Pulmonary Hypertension: Executive Summary. Evian, France : World Health Organization, 1998.
19. Galie N, Olschewski H, Oudiz RJ 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.
20. Galie N, Beghetti M, Gatzoulis MA 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.
21. Galie N, Rubin Lj, 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.
22. Pulido T, Adzerikho I, Channick RN et al. SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369 (9): 809–18.
23. Simonneau G, Rubin LJ, 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.
24. Galie N, Brundage BH, Ghofrani HA 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.
25. Galie N, Humbert M, Vachiery JL 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.
26. Olschevski H, Simonneau G, Galie N et al. Inhaled iloprost in severe pulmonary hypertension. N Engl J Med 2002; 347: 322–9.
27. McLaughlin VV, Oudiz RJ, 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.
28. Lang I, Gomes-Sanches M, Kneussi M et al. Efficacy of long-term subcutaneous treprostinil sodium in pulmonary hypertension. Chest 2006; 129: 1636–43.
29. Badesch DB, Tapson VF, McGoon MD 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.
30. 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.
31. 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.
32. 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).
2. Channick RN, 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.
3. Barst RJ, Rubin LJ, Long WA 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.
4. Galie N, Ghofrani HA, 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.
5. Ghofrani HA, D’Armini AM, 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.
6. Taichman DB, 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.
7. Galie N, Humbert M, Vachiery JL 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.
8. McLaughlin VV, Gaine SP, Howard LS et al. Treatment goals of pulmonary hypertension. J Am CollCardiol 2013; 62 (Suppl. 25): D73–D81.
9. Lewis SZ, Diekemper R, Ornelas J, Casey KR. Methodologies for the development of CHEST guidelines and expert panel reports. Chest 2014; 146 (1): 182–92.
10. 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.
11. Humbert M, Barst RJ, Robbins IM et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24: 353–9.
12. Sitbon O, Jaı¨s X, Savale L et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691–7.
13. Galie` N, Barbera JA, Frost A et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. New Engl J Med 2015; 379 (9): 834–44.
14. Fuster V, Steele PM, Edwards WD et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580–7.
15. 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.
16. Hoeper MM, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12: 1446–9.
17. Sandoval J, Aguirre JS, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682–7.
18. Rich S. Primary Pulmonary Hypertension: Executive Summary. Evian, France : World Health Organization, 1998.
19. Galie N, Olschewski H, Oudiz RJ 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.
20. Galie N, Beghetti M, Gatzoulis MA 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.
21. Galie N, Rubin Lj, 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.
22. Pulido T, Adzerikho I, Channick RN et al. SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369 (9): 809–18.
23. Simonneau G, Rubin LJ, 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.
24. Galie N, Brundage BH, Ghofrani HA 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.
25. Galie N, Humbert M, Vachiery JL 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.
26. Olschevski H, Simonneau G, Galie N et al. Inhaled iloprost in severe pulmonary hypertension. N Engl J Med 2002; 347: 322–9.
27. McLaughlin VV, Oudiz RJ, 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.
28. Lang I, Gomes-Sanches M, Kneussi M et al. Efficacy of long-term subcutaneous treprostinil sodium in pulmonary hypertension. Chest 2006; 129: 1636–43.
29. Badesch DB, Tapson VF, McGoon MD 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.
30. 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.
31. 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.
32. 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).
________________________________________________
2. Channick RN, 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.
3. Barst RJ, Rubin LJ, Long WA 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.
4. Galie N, Ghofrani HA, 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.
5. Ghofrani HA, D’Armini AM, 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.
6. Taichman DB, 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.
7. Galie N, Humbert M, Vachiery JL 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.
8. McLaughlin VV, Gaine SP, Howard LS et al. Treatment goals of pulmonary hypertension. J Am CollCardiol 2013; 62 (Suppl. 25): D73–D81.
9. Lewis SZ, Diekemper R, Ornelas J, Casey KR. Methodologies for the development of CHEST guidelines and expert panel reports. Chest 2014; 146 (1): 182–92.
10. 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.
11. Humbert M, Barst RJ, Robbins IM et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24: 353–9.
12. Sitbon O, Jaı¨s X, Savale L et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691–7.
13. Galie` N, Barbera JA, Frost A et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. New Engl J Med 2015; 379 (9): 834–44.
14. Fuster V, Steele PM, Edwards WD et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580–7.
15. 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.
16. Hoeper MM, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12: 1446–9.
17. Sandoval J, Aguirre JS, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682–7.
18. Rich S. Primary Pulmonary Hypertension: Executive Summary. Evian, France : World Health Organization, 1998.
19. Galie N, Olschewski H, Oudiz RJ 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.
20. Galie N, Beghetti M, Gatzoulis MA 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.
21. Galie N, Rubin Lj, 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.
22. Pulido T, Adzerikho I, Channick RN et al. SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369 (9): 809–18.
23. Simonneau G, Rubin LJ, 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.
24. Galie N, Brundage BH, Ghofrani HA 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.
25. Galie N, Humbert M, Vachiery JL 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.
26. Olschevski H, Simonneau G, Galie N et al. Inhaled iloprost in severe pulmonary hypertension. N Engl J Med 2002; 347: 322–9.
27. McLaughlin VV, Oudiz RJ, 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.
28. Lang I, Gomes-Sanches M, Kneussi M et al. Efficacy of long-term subcutaneous treprostinil sodium in pulmonary hypertension. Chest 2006; 129: 1636–43.
29. Badesch DB, Tapson VF, McGoon MD 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.
30. 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.
31. 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.
32. 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).
Авторы
Т.В.Мартынюк*, И.Е.Чазова
Институт клинической кардиологии им. А.Л.Мясникова ФГБУ Российский кардиологический научно-производственный комплекс Минздрава России. 121552, Россия, Москва, ул. 3-я Черепковская, д. 15а
*trukhiniv@mail.ru
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation. 121552, Russian Federation, Moscow, ul. 3-ia Cherepkovskaia, d. 15a
*trukhiniv@mail.ru
Институт клинической кардиологии им. А.Л.Мясникова ФГБУ Российский кардиологический научно-производственный комплекс Минздрава России. 121552, Россия, Москва, ул. 3-я Черепковская, д. 15а
*trukhiniv@mail.ru
________________________________________________
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation. 121552, Russian Federation, Moscow, ul. 3-ia Cherepkovskaia, d. 15a
*trukhiniv@mail.ru
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