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Возможности комбинированной терапии в лечении пациентов с впервые установленным диагнозом легочной артериальной гипертензии промежуточного риска
Возможности комбинированной терапии в лечении пациентов с впервые установленным диагнозом легочной артериальной гипертензии промежуточного риска
Ляпина И.Н., Зверева Т.Н., Мартынюк Т.В. Возможности комбинированной терапии в лечении пациентов с впервые установленным диагнозом легочной артериальной гипертензии промежуточного риска. Consilium Medicum. 2022;24(10):688–695.
DOI: 10.26442/20751753.2022.10.201875
© ООО «КОНСИЛИУМ МЕДИКУМ», 2022 г.
DOI: 10.26442/20751753.2022.10.201875
DOI: 10.26442/20751753.2022.10.201875
© ООО «КОНСИЛИУМ МЕДИКУМ», 2022 г.
________________________________________________
DOI: 10.26442/20751753.2022.10.201875
Материалы доступны только для специалистов сферы здравоохранения. Авторизуйтесь или зарегистрируйтесь.
Аннотация
Разработки в сфере специфической терапии легочной артериальной гипертензии (ЛАГ) и новых стратегий комбинированной терапии/эскалации методов лечения за последнее десятилетие позволили значимо улучшить контроль заболевания, замедлить его прогрессирование, повысить качество жизни и выживаемость пациентов. В рамках данного обзора проводится детальное обсуждение современных стратегий ЛАГ-специфической терапии, доступной на территории Российской Федерации, у пациентов с классической и неклассической ЛАГ, не достигающих целей лечения и сохраняющих промежуточный риск прогрессирования заболевания/летальности.
Ключевые слова: легочная артериальная гипертензия, промежуточный риск летальности, ЛАГ-специфическая терапия, начальная комбинированная терапия
Keywords: pulmonary arterial hypertension, intermediate risk of mortality, PAH-specific therapy, initial combination therapy
Ключевые слова: легочная артериальная гипертензия, промежуточный риск летальности, ЛАГ-специфическая терапия, начальная комбинированная терапия
________________________________________________
Keywords: pulmonary arterial hypertension, intermediate risk of mortality, PAH-specific therapy, initial combination therapy
Полный текст
Список литературы
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3. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;ehac237. DOI:10.1093/eurheartj/ehac237
4. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension: results fr om a national prospective registry. Ann Intern Med. 1991;115(5):343-9.
DOI:10.7326/0003-4819-115-5-343
5. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival rom time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142:448-56.
DOI:10.1378/chest.11-1460
6. Chazova IE, Arkhipova OA, Martynyuk TV. Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry. Therapeutic Archive. 2019;91(1):24-31. DOI:10.26442/00403660.2019.01.000024
7. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2):871-80. DOI:10.1016/j.ijcard.2012.10.026
8. Dweik RA, Rounds S, Erzurum SC, et al. An official American Thoracic Society Statement: pulmonary hypertension phenotypes. Am J Respir Crit Care Med. 2014;189(3):345-55.
DOI:10.1164/rccm.201311-1954ST
9. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013;62(25):51-9. DOI:10.1016/j.jacc.2013.10.023
10. Radegran G, Kjellstrom B, Ekmehag B, et al. Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014. Scand Cardiovasc J. 2016;50(4):243-50.
DOI:10.1080/14017431.2016.1185532
11. Taran IN, Belevskaya AA, Valieva ZS, et al. “Portrait” of patients with idiopathic pulmonary hypertension and chronic thromboembolic pulmonary hypertension depending on comorbid status: current and prognosis features. PULMONOLOGIYA. 2020;30(4):427-36 (in Russian). DOI:10.18093/0869-0189-2020-30-4-427-436
12. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2):1700740. DOI:10.1183/13993003.00740-2017
13. Hjalmarsson C, Rådegran G, Kylhammar D, et al. Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5):1702310. DOI:10.1183/13993003.02310-2017
14. Taran IN, Valieva ZS, Belevskaya AA, et al. Influence of demographic characteristics on the clinical course and results of therapy in patients with precapillary pulmonary hypertension. Terapevticheskii Arkhiv (Ter. Arkh.). 2021;93(3):283-9 (in Russian). DOI:10.26442/00403660.2021.03.200655
15. Hoeper MM, Boucly A, Sitbon O. Age, risk and outcomes in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5):1800629. DOI:10.1183/13993003.00629-2018
16. Klinger JR, Elliott CG, Levine DJ, et al. Therapy for Pulmonary Arterial Hypertension in Adults Update of the CHEST Guideline and Expert Panel Report. CHEST. 2019;155(3):565-86.
DOI:10.1016/j.chest.2018.11.030
17. Campean IA, Lang IM. Treating pulmonary hypertension in the elderly. Expert Opin Pharmacother. 2020;21(10):1193-200. DOI:10.1080/14656566.2020.1739270
18. Hoeper MM, Pausch C, Grünig E, et al. Temporal trends in pulmonary arterial hypertension: Results from the COMPERA registry. Eur Respir J. 2022;59:2102024.
DOI:10.1183/13993003.02024-2021
19. Quan R, Zhang G, Yu Z, et al. Characteristics, goal-oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry. Respirology. 2022;27(7):517-28. DOI:10.1111/resp.14247
20. Benza RL, Miller DP, Foreman AJ, et al. Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: A Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis. J Heart Lung Transplant. 2015;34(3):356-61. DOI:10.1016/j.healun.2014.09.016
21. Humbert M, Guignabert C, Bonnet S, et al. Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives. Eur Respir J. 2019;53(1):1801887. DOI:10.1183/13993003.01887-2018
22. Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019;53(1):1801904. DOI:10.1183/13993003.01904-2018
23. Martynyuk TV, Chazova IE. Pulmonary arterial hypertension: achievements and realities of modern treatment, a look into the future. Terapevticheskii Arkhiv (Ter. Arkh.). 2021;93(9):1009-17 (in Russian). DOI:10.26442/00403660.2021.09.201013
24. Maron BA. Pulmonary arterial hypertension: Rationale for using multiple vs. single drug therapy. Glob Cardiol Sci Pract. 2020;2020(1):e202008. DOI:10.21542/gcsp.2020.8
25. Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1801889.
DOI:10.1183/13993003.01889-2018
26. Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):1700889.
DOI:10.1183/13993003.00889-2017
27. Hoeper MM, Pittrow D, Opitz C, et al. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018;51(3):1702606. DOI:10.1183/13993003.02606-2017
28. Kylhammar D, Kjellström B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2018;39(47):4175-81. DOI:10.1093/eurheartj/ehx257
29. Hassoun PM. Pulmonary Arterial Hypertension. N Engl J Med. 2021;385(25):2361-76. DOI:10.1056/NEJMra2000348
30. Hoeper MM, Apitz C, Grünig E, et al. Targeted therapy of pulmonary arterial hypertension: Updated recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018;272S:37-45. DOI:10.1016/j.ijcard.2018.08.082
31. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369:809-18.
32. Ghofrani HA, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369:330-40.
33. Sitbon O, Channick R, Kelly C, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373:2522-33.
34. Galiè N, Muller K, Scalise AV, et al. PATENT PLUS: a blinded, randomised and extension study of riociguat plus sildenafil in PAH. Eur Respir J. 2015;45:1314-22.
35. Taran IN, Belevskaya AA, Saidova MA, et al. Initial Riociguat Monotherapy and Transition from Sildenafil to Riociguat in Patients with Idiopathic Pulmonary Arterial Hypertension: Influence on Right Heart Remodeling and Right Ventricular–Pulmonary Arterial Coupling. Lung. 2018;196(6):745-53. DOI:10.1007/s00408-018-0160-4
36. Gratsianskaya SE, Valieva ZS, Martynyuk TV. The achievements of the modern specific therapy of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: focus on the stimulator of soluble guanylate cyclase riociguat. Terapevticheskii arkhiv. 2020;92(9):77-84 (in Russian). DOI:10.26442/00403660.2020.09.000717
37. Hoeper MM, Al-Hiti H, Benza RL, et al; REPLACE investigators. Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial. Lancet Respir Med. 2021;9(6):573-84. DOI:10.1016/S2213-2600(20)30532-4
38. Rahaghi FF, Balasubramanian VP, Bourge RC, et al. Delphi consensus recommendation for optimization of pulmonary hypertension therapy focusing on switching from a phosphodiesterase 5 inhibitor to riociguat. Pulm Circ. 2022;12(2):e12055. DOI:10.1002/pul2.12055
39. Sitbon O, Sattler C, Bertoletti L, et al. Initial dual oral combination therapy in pulmonary arterial hypertension. Eur Respir J. 2016;47(6):1727-36. DOI:10.1183/13993003.02043-2015
40. McLaughlin V, Channick RN, Ghofrani HA, et al. Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J. 2015;46(2):405-13. DOI:10.1183/13993003.02044-2014
41. Vizza CD, Jansa P, Teal S, et al. Sildenafil dosed concomitantly with bosentan for adult pulmonary arterial hypertension in a randomized controlled trial. BMC Cardiovasc Dis. 2017;17(1):239. DOI:10.1186/s12872-017-06743
42. Rinaldi A, Dardi F, Albini A, et al. Haemodynamic and exercise effects of different types of initial oral combination therapy in pulmonary arterial hypertension. Eur Heart J. 2018;39(Suppl. 1):1328.
43. Thornton CS, Helmersen D, Thakrar MV, et al. Early Echocardiographic Improvements with Upfront Riociguat and Ambrisentan Combination Therapy for Incident Pulmonary Arterial Hypertension. J Heart Lung Transplant. 2020;39(4S):S508-09.
44. Weatherald J, Thakrar MV, Varughese RA, et al. Upfront riociguat and ambrisentan combination therapy for newly diagnosed pulmonary arterial hypertension: A prospective open-label trial. J Heart Lung Transplant. 2022;41(5):563-7. DOI:10.1016/j.healun.2022.01.002
45. Sulica R, Sangli S, Chakravarti A, Steiger D. Clinical and hemodynamic benefit of macitentan and riociguat upfront combination in patients with pulmonary arterial hypertension. Pulm Circ. 2019;9(1):2045894019826944. DOI:10.1177/2045894019826944
46. Correale M, Ferraretti A, Monaco I, et al. Endothelin-receptor antagonists in the management of pulmonary arterial hypertension: wh ere do we stand? Vasc Health Risk Manag. 2018;14:253-64. DOI:10.2147/VHRM.S133921
47. Gale S. The evolving treatment landscape of pulmonary arterial hypertension. Am J Manag Care. 2021;27(3 Suppl):S42-52. DOI:10.37765/ajmc.2021.88610
48. Evgenov OV, Pacher P, Schmidt PM, et al. NO-independent stimulators and activators of soluble guanylate cyclase: discovery and therapeutic potential. Nat Rev Drug Discov. 2006;5(9):755-68. DOI:10.1038/nrd2038
49. Wilkens H, Guth A, König J, et al. Effect of inhaled iloprost plus oral sildenafil in patients with primary pulmonary hypertension. Circulation. 2001;104(11):1218-22.
DOI:10.1161/hc3601.096826
50. Ghofrani HA, Wiedemann R, Rose F, et al. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med. 2002;136(7):515-22.
DOI:10.7326/0003-4819-136-7-200204020-00008
51. Резухина Е.А., Мартынюк Т.В., Валиева З.С., и др. Легочная артериальная гипертензия: вызовы и достижения 2021. Евразийский кардиологический журнал.
2022;(1):80-9 [Rezukhina EA, Martynyuk TV, Valieva ZS, et al. Pulmonary arterial hypertension: challenges and achievements 2021. Eurasian heart journal. 2022;(1):80-9 (in Russian)].
DOI:10.38109/2225-1685-2022-1-80-89
52. Chin KM, Sitbon O, Doelberg M, et al. Three- Versus Two-Drug Therapy for Patients With Newly Diagnosed Pulmonary Arterial Hypertension. J Am Coll Cardiol. 2021;78(14):1393-403. DOI:10.1016/j.jacc.2021.07.057
53. Dhoble S, Patravale V, Weaver E, et al. Comprehensive review on novel targets and emerging therapeutic modalities for pulmonary arterial Hypertension. Int J Pharm. 2022;621:121792. DOI:10.1016/j.ijpharm.2022.121792
54. Humbert M, Sitbon O, Yaïci A; French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J. 2010;36(3):549-55. DOI:10.1183/09031936.00057010
55. Shmalts AA, Gorbachevsky SV. Advantages and limitations of initial combination therapy in pulmonary arterial hypertensionpatients in Russia. Terapevticheskii Arkhiv (Ter. Arkh.). 2020;92(12):80-5 (in Russian). DOI:10.26442/00403660.2020.12.000840
2. Чазова И.Е., Мартынюк Т.В., Валиева З.С., и др. Евразийские клинические рекомендации по диагностике и лечению легочной гипертензии. Евразийский кардиологический журнал. 2020;1:78-122 [Chazova IE, Martynyuk TV, Valieva ZS, et al. Eurasian Guidelines for the diagnosis and treatment of pulmonary hypertension. Eurasian Нeart Journal. 2020;1:78-122 (in Russian)]. DOI:10.38109/2225-1685-2020-1-78-122
3. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;ehac237. DOI:10.1093/eurheartj/ehac237
4. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension: results fr om a national prospective registry. Ann Intern Med. 1991;115(5):343-9.
DOI:10.7326/0003-4819-115-5-343
5. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival rom time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142:448-56.
DOI:10.1378/chest.11-1460
6. Чазова И.Е., Архипова О.А., Мартынюк Т.В. Легочная артериальная гипертензия в России: анализ шестилетнего наблюдения по данным Национального регистра. Терапевтический архив. 2019;91(1):24-31 [Chazova IE, Arkhipova OA, Martynyuk TV. Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry. Therapeutic Archive. 2019;91(1):24-31]. DOI:10.26442/00403660.2019.01.000024
7. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2):871-80. DOI:10.1016/j.ijcard.2012.10.026
8. Dweik RA, Rounds S, Erzurum SC, et al. An official American Thoracic Society Statement: pulmonary hypertension phenotypes. Am J Respir Crit Care Med. 2014;189(3):345-55.
DOI:10.1164/rccm.201311-1954ST
9. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013;62(25):51-9. DOI:10.1016/j.jacc.2013.10.023
10. Radegran G, Kjellstrom B, Ekmehag B, et al. Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014. Scand Cardiovasc J. 2016;50(4):243-50.
DOI:10.1080/14017431.2016.1185532
11. Таран И.Н., Белевская А.А., Валиева З.С., и др. «Портрет» пациентов с идиопатической легочной гипертензией и хронической тромбоэмболической легочной гипертензией в зависимости от коморбидного статуса: особенности течения заболевания и прогноз. Пульмонология. 2020;30(4):427-36 [Taran IN, Belevskaya AA, Valieva ZS, et al. “Portrait” of patients with idiopathic pulmonary hypertension and chronic thromboembolic pulmonary hypertension depending on comorbid status: current and prognosis features. PULMONOLOGIYA. 2020;30(4):427-36 (in Russian)]. DOI:10.18093/0869-0189-2020-30-4-427-436
12. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2):1700740. DOI:10.1183/13993003.00740-2017
13. Hjalmarsson C, Rådegran G, Kylhammar D, et al. Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5):1702310. DOI:10.1183/13993003.02310-2017
14. Таран И.Н., Валиева З.С., Белевская А.А., и др. Влияние демографических характеристик на клиническое течение и результаты терапии пациентов с прекапиллярной легочной гипертензией. Терапевтический архив. 2021;93(3):283-9 [Taran IN, Valieva ZS, Belevskaya AA, et al. Influence of demographic characteristics on the clinical course and results of therapy in patients with precapillary pulmonary hypertension. Terapevticheskii Arkhiv (Ter. Arkh.). 2021;93(3):283-9
(in Russian)]. DOI:10.26442/00403660.2021.03.200655
15. Hoeper MM, Boucly A, Sitbon O. Age, risk and outcomes in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5):1800629. DOI:10.1183/13993003.00629-2018
16. Klinger JR, Elliott CG, Levine DJ, et al. Therapy for Pulmonary Arterial Hypertension in Adults Update of the CHEST Guideline and Expert Panel Report. CHEST. 2019;155(3):565-86.
DOI:10.1016/j.chest.2018.11.030
17. Campean IA, Lang IM. Treating pulmonary hypertension in the elderly. Expert Opin Pharmacother. 2020;21(10):1193-200. DOI:10.1080/14656566.2020.1739270
18. Hoeper MM, Pausch C, Grünig E, et al. Temporal trends in pulmonary arterial hypertension: Results from the COMPERA registry. Eur Respir J. 2022;59:2102024.
DOI:10.1183/13993003.02024-2021
19. Quan R, Zhang G, Yu Z, et al. Characteristics, goal-oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry. Respirology. 2022;27(7):517-28. DOI:10.1111/resp.14247
20. Benza RL, Miller DP, Foreman AJ, et al. Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: A Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis. J Heart Lung Transplant. 2015;34(3):356-61. DOI:10.1016/j.healun.2014.09.016
21. Humbert M, Guignabert C, Bonnet S, et al. Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives. Eur Respir J. 2019;53(1):1801887. DOI:10.1183/13993003.01887-2018
22. Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019;53(1):1801904. DOI:10.1183/13993003.01904-2018
23. Мартынюк Т.В., Чазова И.Е. Легочная артериальная гипертензия: достижения и реалии современного лечения, взгляд в будущее. Терапевтический архив. 2021;93(9):1009-17 [Martynyuk TV, Chazova IE. Pulmonary arterial hypertension: achievements and realities of modern treatment, a look into the future. Terapevticheskii Arkhiv (Ter. Arkh.). 2021;93(9):1009-17 (in Russian)]. DOI:10.26442/00403660.2021.09.201013
24. Maron BA. Pulmonary arterial hypertension: Rationale for using multiple vs. single drug therapy. Glob Cardiol Sci Pract. 2020;2020(1):e202008. DOI:10.21542/gcsp.2020.8
25. Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1801889.
DOI:10.1183/13993003.01889-2018
26. Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):1700889.
DOI:10.1183/13993003.00889-2017
27. Hoeper MM, Pittrow D, Opitz C, et al. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018;51(3):1702606. DOI:10.1183/13993003.02606-2017
28. Kylhammar D, Kjellström B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2018;39(47):4175-81. DOI:10.1093/eurheartj/ehx257
29. Hassoun PM. Pulmonary Arterial Hypertension. N Engl J Med. 2021;385(25):2361-76. DOI:10.1056/NEJMra2000348
30. Hoeper MM, Apitz C, Grünig E, et al. Targeted therapy of pulmonary arterial hypertension: Updated recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018;272S:37-45. DOI:10.1016/j.ijcard.2018.08.082
31. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369:809-18.
32. Ghofrani HA, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369:330-40.
33. Sitbon O, Channick R, Kelly C, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373:2522-33.
34. Galiè N, Muller K, Scalise AV, et al. PATENT PLUS: a blinded, randomised and extension study of riociguat plus sildenafil in PAH. Eur Respir J. 2015;45:1314-22.
35. Taran IN, Belevskaya AA, Saidova MA, et al. Initial Riociguat Monotherapy and Transition from Sildenafil to Riociguat in Patients with Idiopathic Pulmonary Arterial Hypertension: Influence on Right Heart Remodeling and Right Ventricular–Pulmonary Arterial Coupling. Lung. 2018;196(6):745-53. DOI:10.1007/s00408-018-0160-4
36. Грацианская С.Е., Валиева З.С., Мартынюк Т.В. Достижения современной специфической терапии легочной артериальной и хронической тромбоэмболической легочной гипертензии: фокус на стимулятор растворимой гуанилатциклазы риоцигуат. Терапевтический архив. 2020;92(9):77-84 [Gratsianskaya SE, Valieva ZS, Martynyuk TV. The achievements of the modern specific therapy of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: focus on the stimulator of soluble guanylate cyclase riociguat. Terapevticheskii arkhiv. 2020;92(9):77-84 (in Russian)]. DOI:10.26442/00403660.2020.09.000717
37. Hoeper MM, Al-Hiti H, Benza RL, et al; REPLACE investigators. Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial. Lancet Respir Med. 2021;9(6):573-84. DOI:10.1016/S2213-2600(20)30532-4
38. Rahaghi FF, Balasubramanian VP, Bourge RC, et al. Delphi consensus recommendation for optimization of pulmonary hypertension therapy focusing on switching from a phosphodiesterase 5 inhibitor to riociguat. Pulm Circ. 2022;12(2):e12055. DOI:10.1002/pul2.12055
39. Sitbon O, Sattler C, Bertoletti L, et al. Initial dual oral combination therapy in pulmonary arterial hypertension. Eur Respir J. 2016;47(6):1727-36. DOI:10.1183/13993003.02043-2015
40. McLaughlin V, Channick RN, Ghofrani HA, et al. Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J. 2015;46(2):405-13. DOI:10.1183/13993003.02044-2014
41. Vizza CD, Jansa P, Teal S, et al. Sildenafil dosed concomitantly with bosentan for adult pulmonary arterial hypertension in a randomized controlled trial. BMC Cardiovasc Dis. 2017;17(1):239. DOI:10.1186/s12872-017-06743
42. Rinaldi A, Dardi F, Albini A, et al. Haemodynamic and exercise effects of different types of initial oral combination therapy in pulmonary arterial hypertension. Eur Heart J. 2018;39(Suppl. 1):1328.
43. Thornton CS, Helmersen D, Thakrar MV, et al. Early Echocardiographic Improvements with Upfront Riociguat and Ambrisentan Combination Therapy for Incident Pulmonary Arterial Hypertension. J Heart Lung Transplant. 2020;39(4S):S508-09.
44. Weatherald J, Thakrar MV, Varughese RA, et al. Upfront riociguat and ambrisentan combination therapy for newly diagnosed pulmonary arterial hypertension: A prospective open-label trial. J Heart Lung Transplant. 2022;41(5):563-7. DOI:10.1016/j.healun.2022.01.002
45. Sulica R, Sangli S, Chakravarti A, Steiger D. Clinical and hemodynamic benefit of macitentan and riociguat upfront combination in patients with pulmonary arterial hypertension. Pulm Circ. 2019;9(1):2045894019826944. DOI:10.1177/2045894019826944
46. Correale M, Ferraretti A, Monaco I, et al. Endothelin-receptor antagonists in the management of pulmonary arterial hypertension: wh ere do we stand? Vasc Health Risk Manag. 2018;14:253-64. DOI:10.2147/VHRM.S133921
47. Gale S. The evolving treatment landscape of pulmonary arterial hypertension. Am J Manag Care. 2021;27(3 Suppl):S42-52. DOI:10.37765/ajmc.2021.88610
48. Evgenov OV, Pacher P, Schmidt PM, et al. NO-independent stimulators and activators of soluble guanylate cyclase: discovery and therapeutic potential. Nat Rev Drug Discov. 2006;5(9):755-68. DOI:10.1038/nrd2038
49. Wilkens H, Guth A, König J, et al. Effect of inhaled iloprost plus oral sildenafil in patients with primary pulmonary hypertension. Circulation. 2001;104(11):1218-22.
DOI:10.1161/hc3601.096826
50. Ghofrani HA, Wiedemann R, Rose F, et al. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med. 2002;136(7):515-22.
DOI:10.7326/0003-4819-136-7-200204020-00008
51. Резухина Е.А., Мартынюк Т.В., Валиева З.С., и др. Легочная артериальная гипертензия: вызовы и достижения 2021. Евразийский кардиологический журнал.
2022;(1):80-9 [Rezukhina EA, Martynyuk TV, Valieva ZS, et al. Pulmonary arterial hypertension: challenges and achievements 2021. Eurasian heart journal. 2022;(1):80-9 (in Russian)].
DOI:10.38109/2225-1685-2022-1-80-89
52. Chin KM, Sitbon O, Doelberg M, et al. Three- Versus Two-Drug Therapy for Patients With Newly Diagnosed Pulmonary Arterial Hypertension. J Am Coll Cardiol. 2021;78(14):1393-403. DOI:10.1016/j.jacc.2021.07.057
53. Dhoble S, Patravale V, Weaver E, et al. Comprehensive review on novel targets and emerging therapeutic modalities for pulmonary arterial Hypertension. Int J Pharm. 2022;621:121792. DOI:10.1016/j.ijpharm.2022.121792
54. Humbert M, Sitbon O, Yaïci A; French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J. 2010;36(3):549-55. DOI:10.1183/09031936.00057010
55. Шмальц А.А., Горбачевский С.В. Возможности и ограничения для начальной комбинированной специфической терапии легочной артериальной гипертензии в Российской Федерации. Терапевтический архив. 2020;92(12):80-5 [Shmalts AA, Gorbachevsky SV. Advantages and limitations of initial combination therapy in pulmonary arterial hypertensionpatients in Russia. Terapevticheskii Arkhiv (Ter. Arkh.). 2020;92(12):80-5 (in Russian)].
DOI:10.26442/00403660.2020.12.000840
________________________________________________
2. Chazova IE, Martynyuk TV, Valieva ZS, et al. Eurasian Guidelines for the diagnosis and treatment of pulmonary hypertension. Eurasian Нeart Journal. 2020;1:78-122 (in Russian). DOI:10.38109/2225-1685-2020-1-78-122
3. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;ehac237. DOI:10.1093/eurheartj/ehac237
4. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension: results fr om a national prospective registry. Ann Intern Med. 1991;115(5):343-9.
DOI:10.7326/0003-4819-115-5-343
5. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival rom time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142:448-56.
DOI:10.1378/chest.11-1460
6. Chazova IE, Arkhipova OA, Martynyuk TV. Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry. Therapeutic Archive. 2019;91(1):24-31. DOI:10.26442/00403660.2019.01.000024
7. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2):871-80. DOI:10.1016/j.ijcard.2012.10.026
8. Dweik RA, Rounds S, Erzurum SC, et al. An official American Thoracic Society Statement: pulmonary hypertension phenotypes. Am J Respir Crit Care Med. 2014;189(3):345-55.
DOI:10.1164/rccm.201311-1954ST
9. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013;62(25):51-9. DOI:10.1016/j.jacc.2013.10.023
10. Radegran G, Kjellstrom B, Ekmehag B, et al. Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014. Scand Cardiovasc J. 2016;50(4):243-50.
DOI:10.1080/14017431.2016.1185532
11. Taran IN, Belevskaya AA, Valieva ZS, et al. “Portrait” of patients with idiopathic pulmonary hypertension and chronic thromboembolic pulmonary hypertension depending on comorbid status: current and prognosis features. PULMONOLOGIYA. 2020;30(4):427-36 (in Russian). DOI:10.18093/0869-0189-2020-30-4-427-436
12. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2):1700740. DOI:10.1183/13993003.00740-2017
13. Hjalmarsson C, Rådegran G, Kylhammar D, et al. Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5):1702310. DOI:10.1183/13993003.02310-2017
14. Taran IN, Valieva ZS, Belevskaya AA, et al. Influence of demographic characteristics on the clinical course and results of therapy in patients with precapillary pulmonary hypertension. Terapevticheskii Arkhiv (Ter. Arkh.). 2021;93(3):283-9 (in Russian). DOI:10.26442/00403660.2021.03.200655
15. Hoeper MM, Boucly A, Sitbon O. Age, risk and outcomes in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5):1800629. DOI:10.1183/13993003.00629-2018
16. Klinger JR, Elliott CG, Levine DJ, et al. Therapy for Pulmonary Arterial Hypertension in Adults Update of the CHEST Guideline and Expert Panel Report. CHEST. 2019;155(3):565-86.
DOI:10.1016/j.chest.2018.11.030
17. Campean IA, Lang IM. Treating pulmonary hypertension in the elderly. Expert Opin Pharmacother. 2020;21(10):1193-200. DOI:10.1080/14656566.2020.1739270
18. Hoeper MM, Pausch C, Grünig E, et al. Temporal trends in pulmonary arterial hypertension: Results from the COMPERA registry. Eur Respir J. 2022;59:2102024.
DOI:10.1183/13993003.02024-2021
19. Quan R, Zhang G, Yu Z, et al. Characteristics, goal-oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry. Respirology. 2022;27(7):517-28. DOI:10.1111/resp.14247
20. Benza RL, Miller DP, Foreman AJ, et al. Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: A Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis. J Heart Lung Transplant. 2015;34(3):356-61. DOI:10.1016/j.healun.2014.09.016
21. Humbert M, Guignabert C, Bonnet S, et al. Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives. Eur Respir J. 2019;53(1):1801887. DOI:10.1183/13993003.01887-2018
22. Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019;53(1):1801904. DOI:10.1183/13993003.01904-2018
23. Martynyuk TV, Chazova IE. Pulmonary arterial hypertension: achievements and realities of modern treatment, a look into the future. Terapevticheskii Arkhiv (Ter. Arkh.). 2021;93(9):1009-17 (in Russian). DOI:10.26442/00403660.2021.09.201013
24. Maron BA. Pulmonary arterial hypertension: Rationale for using multiple vs. single drug therapy. Glob Cardiol Sci Pract. 2020;2020(1):e202008. DOI:10.21542/gcsp.2020.8
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Авторы
И.Н. Ляпина*1,2, Т.Н. Зверева1,2, Т.В. Мартынюк3,4
1 ФГБНУ «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», Кемерово, Россия;
2 ФГБОУ ВО «Кемеровский государственный университет» Минздрава России, Кемерово, Россия;
3 ФГБУ «Национальный медицинский исследовательский центр кардиологии им. акад. Е.И. Чазова» Минздрава России, Москва, Россия;
4 ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова», Москва, Россия
*zaviirina@mail.ru
1 Research Institute for Complex Issues of Cardiovascular Diseases, Kemerovo, Russia;
2 Kemerovo State Medical University, Kemerovo, Russia;
3 Chazov National Medical Research Center of Cardiology, Moscow, Russia;
4 Pirogov Russian National Research Medical University, Moscow, Russia
*zaviirina@mail.ru
1 ФГБНУ «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», Кемерово, Россия;
2 ФГБОУ ВО «Кемеровский государственный университет» Минздрава России, Кемерово, Россия;
3 ФГБУ «Национальный медицинский исследовательский центр кардиологии им. акад. Е.И. Чазова» Минздрава России, Москва, Россия;
4 ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова», Москва, Россия
*zaviirina@mail.ru
________________________________________________
1 Research Institute for Complex Issues of Cardiovascular Diseases, Kemerovo, Russia;
2 Kemerovo State Medical University, Kemerovo, Russia;
3 Chazov National Medical Research Center of Cardiology, Moscow, Russia;
4 Pirogov Russian National Research Medical University, Moscow, Russia
*zaviirina@mail.ru
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