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На пути оптимизации подходов к лечению хронической тромбоэмболической легочной гипертензии
Мартынюк Т.В., Наконечников С.Н., Чазова И.Е. На пути оптимизации подходов к лечению хронической тромбоэмболической легочной гипертензии. Consilium Medicum. 2016; 18 (5): 53–58. DOI: 10.26442/2075-1753_2016.5.53-58
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Martyniuk T.V., Nakonechnikov S.N., Chazova I.E. On the way to optimization approach for the treatment of chronic thromboembolic pulmonary hypertension. Consilium Medicum. 2016; 18 (5): 53–58. DOI: 10.26442/2075-1753_2016.5.53-58
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Аннотация
Хроническая тромбоэмболическая легочная гипертензия (ХТЭЛГ) является особой, потенциально излечимой формой патологии. Однако при невозможности проведения хирургического лечения, при персистирующей или резидуальной форме ХТЭЛГ после тромбэндартерэктомии больным назначается специфическая терапия, применяемая при легочной артериальной гипертензии (ЛАГ). В 2014 г. в нашей стране для лечения больных с неоперабельными и резидульными формами ХТЭЛГ был одобрен первый препарат из класса стимуляторов растворимой гуанилатциклазы риоцигуат. В Российских рекомендациях по диагностике и лечению ХТЭЛГ 2015 г. он рассматривается в качестве препарата первого выбора. В качестве препарата 2-й линии для постоянного лечения пациентов с неоперабельной ХТЭЛГ рекомендуется ингаляционный илопрост. Препаратами 3-го ряда являются антагонисты рецепторов эндотелина и ингибиторы фосфодиэстеразы 5-го типа. Общие мероприятия и стандартное лечение при ХТЭЛГ не отличаются от тактики у пациентов с ЛАГ. Прием оральных антикоагулянтов у пациентов с ХТЭЛГ является обязательным на протяжении всей жизни с достижением целевого международного нормализованного отношения 2,5–3,5. Препаратом выбора является антагонист витамина К варфарин. Новые антикоагулянты не рекомендуются в связи с отсутствием доказательной базы. В качестве альтернативы при непереносимости варфарина или повышенном риске кровотечений рекомендуются низкомолекулярные гепарины.
Ключевые слова: хроническая тромбоэмболическая легочная гипертензия, медикаментозная терапия, риоцигуат, стимуляторы растворимой гуанилатциклазы.
Ключевые слова: хроническая тромбоэмболическая легочная гипертензия, медикаментозная терапия, риоцигуат, стимуляторы растворимой гуанилатциклазы.
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Chronic thromboembolic pulmonary hypertension (CTEPH) is unique and potentially curable pathology. However, if surgery is not possible in patients with persistent or residual CTEPH after thromboendarterectomy the specific therapy that is usually used in pulmonary arterial hypertension (PAH) can be prescribed. The first drug from the soluble guanylate cyclase promoters known as riociguat was approved in our country for the treatment of patients with inoperable and residual CTEPH in 2014. Riociguat is discussed as the drug of first choice in the Russian recommendations for the diagnosis and treatment of CTEPH 2015. Inhaled iloprost is recommended as 2nd-line permanent treatment in patients with inoperable CTEPH. Endothelin receptor antagonists and phosphodiesterase type 5 inhibitors are the third-line drugs. Common arrangements and standard treatment in case of CTEPH patients do not differ from the treatment approaches in PAH patients. CTEPH patients should take oral anticoagulants for the rest of life to maintain a target INR of 2.5–3.5. Drug of choice is warfarin – vitamin K antagonist. New anticoagulants are not recommended due to absence of statistical significance. Low molecular weight heparins are recommended as an alternative in case of intolerance to warfarin or increased risk of developing bleeding.
Key words: chronic thromboembolic pulmonary hypertension, drug therapy, riociguat, soluble guanylate cyclase promoters.
Полный текст
Список литературы
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11. Wilkens H, Lang I, Behr J et al. Chronic thromboembolic pulmonary hypertension (CTEPH): updated recommendations of the Cologne Consensus Conference 2011. Int J Cardiol 2011; 154: S54–60.
12. Ghofrani H-A, D’Armini AM, Grimminger F et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369: 319–29.
13. Ghofrani H-A, Hoeper MM, Halank M et al. Riociguat for chronic Thromboembolic pulmonary hypertension and pulmonary arterial hypertension: A phase II study. Eur Respir J 2010; 36: 792–9.
14. Simonneau G, D’Armini AM, Ghofrani H-A et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J 2014. DOI: 10.1183/09031936.00087114.
15. Olschewski H, Simonneau G, Galiè N et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347 (5): 322–9.
16. Мершин К.В., Мартынюк Т.В. Место медикаментозной терапии в лечении хронической тромбоэмболической легочной гипертензии. Евразийский кардиол. журн. 2014; 1: 113–8. / Mershin K.V., Martyniuk T.V. Mesto medikamentoznoi terapii v lechenii khronicheskoi tromboembolicheskoi legochnoi gipertenzii. Evraziiskii kardiol. zhurn. 2014; 1: 113–8. [in Russian]
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21. Jais X, D’Armini AM, Jansa P et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in inoperable forms of chronic thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol 2008; 52: 2127–34.
22. Reichenberger F, Voswinckel R, Enke B et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J 2007; 30: 922–7.
2. Kim NH, Delcroix M, Jenkins DP et al. Chronic Thromboembolic Pulmonary Hypertension. J Am Coll Cardiol 2013; 62 (Suppl): 92–9.
3. Pengo V, Lensing AW, Prins MH et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350: 2257–64.
4. Tapson VF, Humbert M. Incidence and Prevalence of Chronic Thromboembolic Pulmonary Hypertension. Proc Am Thorac Soc 2006; 3: 564–7.
5. Riedel M, Stanek V, Widimsky J et al. Long-term follow-up of patients with pulmonary thromboembolism: late prognosis and evolusion of hemodynamic and respiratory data. Chest 1982; 81: 151–8.
6. Pepke-Zaba J, Delcroix M, Lang I et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 2011; 124: 1973–81.
7. Galiè N, Hoeper MM, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37 (1): 67–119.
8. Мершин К.В., Акчурин Р.С. Хроническая тромбоэмболическая легочная гипертензия. В кн.: Легочная гипертензия. Под ред. И.Е.Чазовой, Т.В.Мартынюк. М.: Практика, 2015; с. 563–602. / Mershin K.V., Akchurin R.S. Khronicheskaia tromboembolicheskaia legochnaia gipertenziia. V kn.: Legochnaia gipertenziia. Pod red. I.E.Chazovoi, T.V.Martyniuk. M.: Praktika, 2015; s. 563–602. [in Russian]
9. Hoeper MM, Mayer E, Simonneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation 2006; 113: 2011–20.
10. Fedullo PF, Auger WR, Kerr KM et al. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001; 345: 1465–72.
11. Wilkens H, Lang I, Behr J et al. Chronic thromboembolic pulmonary hypertension (CTEPH): updated recommendations of the Cologne Consensus Conference 2011. Int J Cardiol 2011; 154: S54–60.
12. Ghofrani H-A, D’Armini AM, Grimminger F et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369: 319–29.
13. Ghofrani H-A, Hoeper MM, Halank M et al. Riociguat for chronic Thromboembolic pulmonary hypertension and pulmonary arterial hypertension: A phase II study. Eur Respir J 2010; 36: 792–9.
14. Simonneau G, D’Armini AM, Ghofrani H-A et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J 2014. DOI: 10.1183/09031936.00087114.
15. Olschewski H, Simonneau G, Galiè N et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347 (5): 322–9.
16. Мершин К.В., Мартынюк Т.В. Место медикаментозной терапии в лечении хронической тромбоэмболической легочной гипертензии. Евразийский кардиол. журн. 2014; 1: 113–8. / Mershin K.V., Martyniuk T.V. Mesto medikamentoznoi terapii v lechenii khronicheskoi tromboembolicheskoi legochnoi gipertenzii. Evraziiskii kardiol. zhurn. 2014; 1: 113–8. [in Russian]
17. Cabrol S, Souza R, Jais X et al. Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2007; 26: 357–62.
18. Skoro-Sajer N, Bonderman D, Wiesbauer F et al. Treprostinil for severe inoperable chronic thromboembolic pulmonary hypertension. Thromb Haemost 2007; 5: 483–9.
19. Reesink HJ, Surie S, Kloek JJ et al. Bosentan as a bridge to pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 2009; 139: 85–91.
20. Bonderman D, Nowotny R, Skoro-Sajer N et al. Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest 2005; 128: 2599–603.
21. Jais X, D’Armini AM, Jansa P et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in inoperable forms of chronic thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol 2008; 52: 2127–34.
22. Reichenberger F, Voswinckel R, Enke B et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J 2007; 30: 922–7.
________________________________________________
1. Chazova I.E., Avdeev S.N., Tsareva N.A. i dr. Klinicheskie rekomendatsii po diagnostike i lecheniiu legochnoi gipertenzii. Terapevt. arkh. 2014; 9: 4–23. [in Russian]
2. Kim NH, Delcroix M, Jenkins DP et al. Chronic Thromboembolic Pulmonary Hypertension. J Am Coll Cardiol 2013; 62 (Suppl): 92–9.
3. Pengo V, Lensing AW, Prins MH et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350: 2257–64.
4. Tapson VF, Humbert M. Incidence and Prevalence of Chronic Thromboembolic Pulmonary Hypertension. Proc Am Thorac Soc 2006; 3: 564–7.
5. Riedel M, Stanek V, Widimsky J et al. Long-term follow-up of patients with pulmonary thromboembolism: late prognosis and evolusion of hemodynamic and respiratory data. Chest 1982; 81: 151–8.
6. Pepke-Zaba J, Delcroix M, Lang I et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 2011; 124: 1973–81.
7. Galiè N, Hoeper MM, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37 (1): 67–119.
8. Mershin K.V., Akchurin R.S. Khronicheskaia tromboembolicheskaia legochnaia gipertenziia. V kn.: Legochnaia gipertenziia. Pod red. I.E.Chazovoi, T.V.Martyniuk. M.: Praktika, 2015; s. 563–602. [in Russian]
9. Hoeper MM, Mayer E, Simonneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation 2006; 113: 2011–20.
10. Fedullo PF, Auger WR, Kerr KM et al. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001; 345: 1465–72.
11. Wilkens H, Lang I, Behr J et al. Chronic thromboembolic pulmonary hypertension (CTEPH): updated recommendations of the Cologne Consensus Conference 2011. Int J Cardiol 2011; 154: S54–60.
12. Ghofrani H-A, D’Armini AM, Grimminger F et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369: 319–29.
13. Ghofrani H-A, Hoeper MM, Halank M et al. Riociguat for chronic Thromboembolic pulmonary hypertension and pulmonary arterial hypertension: A phase II study. Eur Respir J 2010; 36: 792–9.
14. Simonneau G, D’Armini AM, Ghofrani H-A et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J 2014. DOI: 10.1183/09031936.00087114.
15. Olschewski H, Simonneau G, Galiè N et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347 (5): 322–9.
16. Mershin K.V., Martyniuk T.V. Mesto medikamentoznoi terapii v lechenii khronicheskoi tromboembolicheskoi legochnoi gipertenzii. Evraziiskii kardiol. zhurn. 2014; 1: 113–8. [in Russian]
17. Cabrol S, Souza R, Jais X et al. Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2007; 26: 357–62.
18. Skoro-Sajer N, Bonderman D, Wiesbauer F et al. Treprostinil for severe inoperable chronic thromboembolic pulmonary hypertension. Thromb Haemost 2007; 5: 483–9.
19. Reesink HJ, Surie S, Kloek JJ et al. Bosentan as a bridge to pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 2009; 139: 85–91.
20. Bonderman D, Nowotny R, Skoro-Sajer N et al. Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest 2005; 128: 2599–603.
21. Jais X, D’Armini AM, Jansa P et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in inoperable forms of chronic thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol 2008; 52: 2127–34.
22. Reichenberger F, Voswinckel R, Enke B et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J 2007; 30: 922–7.
Авторы
Т.В.Мартынюк*, С.Н.Наконечников, И.Е.Чазова
ФГБУ Российский кардиологический научно-производственный комплекс Минздрава России. 121552, Россия, Москва, ул. 3-я Черепковская, д. 15а
*trukhiniv@mail.ru
ФГБУ Российский кардиологический научно-производственный комплекс Минздрава России. 121552, Россия, Москва, ул. 3-я Черепковская, д. 15а
*trukhiniv@mail.ru
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T.V.Martyniuk*, S.N.Nakonechnikov, I.E.Chazova
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
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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