Евразийские рекомендации по диагностике и лечению лёгочной гипертензии (2023)

лёгочная гипертензия, лёгочная артериальная гипертензия, хроническая тромбоэмболическая лёгочная гипертензия, диагностика, хирургическое лечение, эндоваскулярное лечение, специфическая терапия, клинические рекомендации

1. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019; 53:1801913. https://doi.org/10.1183/13993003.01913-2018

2. Humbert M, Kovacs G, Hoeper MM, et al. ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension Developed by 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 for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG). Eur Heart J 2022; 43(38):3618–3731. http://dx.doi.org/10.1093/eurheartj/ehac237

3. Чазова И.Е., Авдеев С.Н., Царева Н.А., др. Клинические рекомендации по диагностике и лечению лёгочной гипертензии. Терапевтический архив 2014;9:4–23.

4. Мартынюк Т.В. Лёгочная гипертензия: диагностика и лечение. Москва, 2018. Серия Библиотека ФГБУ «НМИЦ кардиологии» Минздрава России. ISBN 978-5-6040008-0-9

5. 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):903-975. http://dx.doi.org/10.1183/13993003.01177-2015

6. Чазова И.Е., Мартынюк Т.В., Филиппов Е.В., др. Клинические рекомендации по диагностике и лечению хронической тромбоэмболической лёгочной гипертензии. (I часть). Терапевтический архив 2016; 88(9):90-101. https://doi.org/10.17116/terarkh201688990-101

7. Kim N.H., Delcroix M., Jais X. et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J 2019; 53:1801915. https://doi.org/10.1183/13993003.01915-2018

8. Wilkens H, Konstantinides S, Lang IM, Bunck AC, Gerges M, Gerhardt F, Grgic A, Grohé C, Guth S, Held M, Hinrichs JB, Hoeper MM, Klepetko W, Kramm T, Krüger U, Lankeit M, Meyer BC, Olsson KM, Schäfers HJ, Schmidt M, Seyfarth HJ, Ulrich S, Wiedenroth CB, Mayer E. Chronic thromboembolic pulmonary hypertension (CTEPH): Updated Recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018 Dec 1;272S:69-78. https://doi.org/10.1016/j.ijcard.2018.08.079

9. Чазова И.Е., Мартынюк Т.В., Валиева З.С., др. Евразийские клинические рекомендации по диагностике и лечению лёгочной гипертензии (2019). Евразийский Кардиологический Журнал 2020; 1:78-122. https://doi.org/10.38109/2225-1685-2020-1-78-122

10. Чазова И.Е., Мартынюк Т.В. Лёгочная гипертензия. – Москва. Практика. 2015; 928с.

11. Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005;111:3105–3111. https://doi.org/10.1161/circulationaha.104.488486

12. Чазова И.Е., Архипова О.А., Мартынюк Т.В. Лёгочная артериальная гипертензия в России: анализ шестилетнего наблюдения по данным Национального регистра. Терапевтический архив 2019; 91(1):10–31. https://doi.org/10.26442/00403660.2019.01.000024

13. Chazova IE, Martynyuk TV, Valieva ZS, et al. Clinical and Instrumental Characteristics of Newly Diagnosed Patients with Various Forms of Pulmonary Hypertension according to the Russian National Registry. BioMed Research International. 2020:6836973. PMID: 32626754. https://doi.org/10.1155/2020/6836973

14. Чазова И.Е., Горбачевский С.В., Мартынюк Т.В., др. Евразийские рекомендации по диагностике и лечению лёгочной гипертензии, ассоциированной с врождёнными пороками сердца у взрослых (2021). Евразийский кардиологический журнал. 2022;2:6-70. https://doi.org/10.38109/2225-1685-2022-2-6-7

15. Sitbon O, Lascoux-Combe C, Delfraissy JF, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med 2008;177:108–113. https://doi.org/10.1164/rccm.200704-541oc

16. Montani D, Price LC, Dorfmuller P, et al. Pulmonary veno-occlusive disease. Eur Respir J 2009;33:189–200. https://doi.org/10.1183/09031936.00090608

17. Rosenkranz S, Gibbs JS, Wachter R, De Marco T, Vonk-Noordegraaf A, Vachiéry JL. Left ventricular heart failure and pulmonary hypertension. Eur Heart J. 2016 Mar 21;37(12):942-54. https://doi.org/10.1093/eurheartj/ehv512

18. Мартынюк Т.В. «Лёгочная гипертензия вследствие патологии левых отделов сердца». Глава в руководстве для врачей «Лёгочная гипертензия» под ред. Авдеева С.Н. (2 издание). ГЭОТАР-МЕДИА, Москва, 2019. С. 213-254.

19. Chaouat A, Bugnet AS, Kadaoui N, et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2005;172:189-194. https://doi.org/10.1164/rccm.200401-006oc

20. Maripov A, Mamazhakypov A, Karagulova G, et al. High altitude pulmonary hypertension with severe right ventricular dysfunction. Int J Cardiol 2013; 168(3):e89–e90. https://doi.org/10.1016/j.ijcard.2013.07.129

21. Leon-Velarde F, Maggiorini M, Reeves JT, et al. Consensus statement on chronic and subacute high altitude diseases. High Alt Med Biol 2005; 6:147-157. https://doi.org/10.1089/ham.2005.6.147

22. Авдеев С.Н., Барбараш О.Л., Баутин А.Е., Волков А.В., Веселова Т.Н., Галявич А.С., Гончарова Н.С., Горбачевский С.В., Данилов Н.М., Еременко А.А., Мартынюк Т.В., Моисеева О.М., Саидова М.А., Сергиенко В.Б., Симакова М.А., Стукалова О.В., Чазова И.Е., Чернявский А.М., Шалаев С.В., Шмальц А.А., Царева Н.А. Легочная гипертензия, в том числе хроническая тромбоэмболическая легочная гипертензия. Клинические рекомендации 2020. Российский кардиологический журнал. 2021;26(12):4683. https://doi.org/10.15829/1560-4071-2021-4683

23. Montani D, Girerd B, Jais X, et al. Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation. Eur Respir J 2020; 58: 2004229. https://doi.org/10.1183/13993003.04229-2020

24. Kovacs G, Berghold A, Scheidl S, et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 2009; 34(4):888–894. https://doi.org/10.1183/09031936.00145608

25. Pieske B, Tschope C, de Boer RA, et al. How to diagnose heart failure with preserved ejection fraction: the HFA-PEFF diagnostic algorithm: a consensus recommendation from the Heart Failure Association (HFA) of the European Society of Cardiology (ESC). Eur Heart J 2019; 40: 3297–3317. https://doi.org/10.1093/eurheartj/ehz641

26. Maron BA, Brittain EL, Hess E, et al. Pulmonary vascular resistance and clinical outcomes in patients with pulmonary hypertension: a retrospective cohort study. Lancet Respir Med 2020; 8:873–884. https://doi.org/10.1016/s2213-2600(20)30317-9

27. Frost A, Badesch D, Simon JR, et al. Diagnosis of pulmonary hypertension. Eur Respir J 2019; 53:1801904. https://doi.org/10.1183/13993003.01904-2018

28. Morrell N, Adnot S, Archer S, et al. Cellular and molecular basis of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54:S20-S31. https://doi.org/10.1016/j.jacc.2009.04.018

29. Olschewski A, Berghausen EM, Eichstaedt CA, et al. Pathobiology, pathology and genetics of pulmonary hypertension: Update from the Cologne Consensus Conference 2018. Int J Cardiol 2018; 272S:4–10. https://doi.org/10.1016/j.ijcard.2018.09.070

30. Rosenkranz S, Lang IM, Blindt R, et al. Pulmonary hypertension associated with left heart disease: Updated Recommendations of the Cologne Consensus Conference 2018. Int J Cardiol 2018; 272S:53–62. https://doi.org/10.1016/j.ijcard.2018.08.080

31. Hurdman J, Condliffe R, Elliot CA, et al. Pulmonary hypertension in COPD: results from the ASPIRE registry. Eur Respir J 2013; 41:1292–1301. https://doi.org/10.1183/09031936.00079512

32. Nathan SD, Barbera JA, Gaine SP, et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J 2019; 53:1801914. https://doi.org/10.1183/13993003.01914-2018

33. Наконечников С.Н., Зыков К.А., Мартынюк Т.В., др. Профиль провоспалительных маркеров у пациентов с различными формами лёгочной гипертензии. Системные гипертензии 2010; 3:61-67. https://doi.org/10.26442/SG28888

34. Simonneau G, Torbicki A, Dorfmüller P, Kim N. The pathophysiology of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26(143):160112. https://doi.org/10.1183/16000617.0112-2016

35. Бокерия Л.А., Горбачевский С.В., Шмальц А.А. Лёгочная артериальная гипертензия, ассоциированная с врождёнными пороками сердца у взрослых. Методическое пособие./ НЦССХ им. А. Н. Бакулева РАМН, 2016 г. ISBN: 978-5-7982-0368-0

36. Hoeper MM, Humbert M, Souza R, et al. A global view of pulmonary hypertension. Lancet Respir Med 2016; 4:306–322. https://doi.org/10.1016/s2213-2600(15)00543-3

37. Leber L, Beaudet A, Muller A. Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review. Pulm Circ 2021; 11:2045894020977300. https://doi.org/10.1177/2045894020977300

38. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107(2):216–223. https://doi.org/10.7326/0003-4819-107-2-216

39. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010; 122 (2):156–163. https://doi.org/10.1161/circulationaha.109.911818

40. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010; 137(2):376–387. https://doi.org/10.1378/chest.09-1140

41. 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):871–880. https://doi.org/10.1016/j.ijcard.2012.10.026

42. Чазова И.Е., Архипова О.А., Валиева З.С., др. Лёгочная гипертензия в России: первые результаты национального регистра. Терапевтический архив 2014; 86(9):56-64.

43. Naeije R. Pulmonary hypertension at high altitude. Eur Respir J 2019; 53(6):1900985. https://doi.org/10.1183/13993003.00985-2019

44. Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001; 345:1465-1472. https://doi.org/10.1056/nejmra010902

45. Delcroix M, Torbicki A, Gopalan D, et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J 2021; 57(6):2002828. https://doi.org/10.1183/13993003.02828-2020

46. Kramm T, Wilkens H, Fuge J, Schäfers HJ, Guth S, Wiedenroth CB, Weingard B, Huscher D, Pittrow D, Cebotari S, Hoeper MM, Mayer E, Olsson KM. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol. 2018 Jul;107(7):548-553. https://doi.org/10.1007/s00392-018-1215-5

47. Чазова И.Е., Валиева З.С., Наконечников С.Н. др. Особенности клинико-функционального и гемодинамического профиля, лекарственной терапии и оценка прогноза у пациентов с неоперабельной хронической тромбоэмболической и идиопатической лёгочной гипертензией по данным Российского регистра. Терапевтический архив 2019; 91(9):77-87. https://doi.org/10.26442/00403660.2019.09.000343

48. Boucly A, Cottin V, Nunes H, et al. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension. Eur Respir J 2017; 50:1700465. https://doi.org/10.1183/13993003.00465-2017

49. Чазова И.Е., Мартынюк Т.В., Валиева З.С., Азизов В.А., Акчурин Р.С., Аншелес А.А., Васильцева О.Я., Веселова Т.Н., Галявич А.С., Горбачевский С.В., Данилов Н.М., Едемский А.Г., Зелвеян П.А., Лазарева И.В., Матчин Ю.Г., Мершин К.В., Мукаров М.А., Наконечников С.Н., Саидова М.А., Сарыбаев А.Ш., Сергиенко В.Б., Стукалова О.В., Филиппов Е.В., Чернявский А.М., Чернявский М.А., Шалаев С.В., Шмальц А.А. Евразийские рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (2020). Евразийский Кардиологический Журнал. 2021;(1):6-43. https://doi.org/10.38109/2225-1685-2021-1-6-43

50. Алокова Ф.Х., Блинова Е.В., Сахнова Т.А., Архипова О.А., Рябыкина Г.В., Саидова М.А., Мартынюк Т.В., Чазова И.Е. Изменения электрокардиограммы в 12 отведениях у больных с идиопатической легочной гипертензией. Медицинский алфавит. 2018;4(36):29-35.

51. Klok FA, Surie S, Kempf T, et al. A simple non-invasive diagnostic algorithm for ruling out chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism. Thromb Res 2011;128:21-26. https://doi.org/10.1016/j.thromres.2011.03.004

52. Tongers J, Schwerdtfeger B, Klein G, et al. Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J 2007; 153:127-132. https://doi.org/10.1016/j.ahj.2006.09.008

53. Kamphuis VP, Haeck ML, Wagner GS, et al. Electrocardiographic detection of right ventricular pressure overload in patients with suspected pulmonary hypertension. J Electrocardiol 2014; 47(2):175-182. https://doi.org/10.1016/j.jelectrocard.2013.10.010

54. Сахнова Т.А., Блинова Е.В., Белевская А.А., др. Сопоставление интегральных показателей векторкардиограммы с данными эхокардиографического исследования у больных идиопатической и хронической тромбоэмболической лёгочной гипертензией. Терапевтический архив 2019;3:11-16. https://doi.org/10.26442/00403660.2019.03.000043

55. Remy-Jardin M, Ryerson CJ, Schiebler ML, et al. Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society. Eur Respir J 2021;57:2004455. https://doi.org/10.1183/13993003.04455-2020

56. Коробкова И.З., Лазуткина В.К., Низовцова Л.А., Ридэн Т.В. Методические аспекты рентгенологической оценки лёгочной гипертензии. ВРР 2015;4:45-53. https://doi.org/10.20862/0042-4676-2015-0-4-14-21

57. Ascha M, Renapurkar RD, Tonelli AR. A review of imaging modalities in pulmonaryhypertension. Ann Thorac Med 2017;12:61-73. https://doi.org/10.4103/1817-1737.203742

58. Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol 2003; 41:1028-1035. https://doi.org/10.1016/s0735-1097(02)02964-9

59. Rudski LG, Lai WW, Afilalo J, et al. Guidelines for the echocardiographic assessment of the right heart in adults:a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 2010; 23:685–713. https://doi.org/10.1016/j.echo.2010.05.010

60. Белевская А.А., Дадачева З.Х., Саидова М.А., др. Возможности эхокардиографии в диагностике лёгочной гипертензии и оценке ремоделирования сердца. Лечебное дело 2015;1:111-121.

61. Rich JD, Shah SJ, Swamy RS et al. Inaccuracy of Doppler echocardiographic estimates of pulmonary artery pressures in patients with pulmonary hypertension: implications for clinical practice. Chest 2011; 139(5):988-993. https://doi.org/10.1378/chest.10-1269

62. Finkelhor RS, Lewis SA, Pillai D. Limitations and strengths of doppler/echo pulmonary artery systolic pressure-right heart catheterization correlations: a systematic literature review. Echocardiography 2015;32(1):10-18. https://doi.org/10.1111/echo.12594

63. Magnino C, Omede P, Avenatti E, et al. Inaccuracy of right atrial pressure estimates through inferior vena cava indices. Am J Cardiol 2017; 120 (9):1667-1673. https://doi.org/10.1016/j.amjcard.2017.07.069

64. Berger M, Haimowitz A, Van Tosh A, et al. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol 1985; 6 (2):359-65. https://doi.org/10.1016/s0735-1097(85)80172-8

65. Greiner S, Jud A, Aurich M et al. Reliability of noninvasive assessment of systolic pulmonary artery pressure by Doppler echocardiography compared to right heart catheterization: analysis in a large patient population. J Am Heart Assoc. 2014; 3(4): e001103. https://doi.org/10.1161/jaha.114.001103

66. Ni JR, Yan PJ, Liu SD, et al. Diagnostic accuracy of transthoracic echocardiography for pulmonary hypertension: a systematic review and meta-analysis. BMJ Open 2019; 22;9(12):e033084. https://doi.org/10.1136/bmjopen-2019-033084

67. Amsallem M, Sternbach JM, Adigopula S, et al. Addressing the Controversy of Estimating Pulmonary Arterial Pressure by Echocardiography. J Am Soc Echocardiogr 2016; 29 (2):93-102 https://doi.org/10.1016/j.echo.2015.11.001

68. Willis J, Henry F, Wild C, et al. Echocardiographic probability of pulmonary hypertension: a real world audit comparing 2015 and 2022 ESC guidelines. Abstract presented at ESC Congress 2023. Eur Heart J 2023; Vol. 44, Issue Supplement_October 2023; in press.

69. D'Alto M, Romeo E, Argiento P, et al. Accuracy and precision of echocardiography versus right heart catheterization for the assessment of pulmonary hypertension. Int J Cardiol 2013;168(4):4058-62. https://doi.org/10.1016/j.ijcard.2013.07.005

70. Dimopoulos K, Condliffe R, Tulloh RMR, et al. Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease: JACC Review Topic of the Week. J Am Coll Cardiol 2018; 72(22):2778-2788. https://doi.org/10.1016/j.jacc.2018.08.2201

71. Nagueh SF, Middleton KJ, Kopelen HA, et al. Doppler tissue imaging: a noninvasive technique for evaluation of left ventricular relaxation and estimation of filling pressures. J Am Coll Cardiol 1997; 30(6):1527-33. https://doi.org/10.1016/s0735-1097(97)00344-6

72. Todaro MC, Carerj S, Zito C, et al. Echocardiographic evaluation of right ventricular-arterial coupling in pulmonary hypertension. Am J Cardiovasc Dis. 2020;10(4):272-283. PMID: 33224574

73. Vizza CD, Lang IM, Badagliacca R, et al. Aggressive Afterload Lowering to Improve the Right Ventricle: A New Target for Medical Therapy in Pulmonary Arterial Hypertension? Am J Respir Crit Care Med 2022; 205(7):751-760. https://doi.org/10.1164/rccm.202109-2079pp

74. Wright LM, Dwyer N, Celermajer D, et al. Follow-Up of Pulmonary Hypertension With Echocardiography. JACC Cardiovasc Imaging 2016; 9 (6):733-46. https://doi.org/10.1016/j.jcmg.2016.02.022

75. Wright L, Dwyer N, Wahi S, Marwick TH. Relative Importance of Baseline and Longitudinal Evaluation in the Follow-Up of Vasodilator Therapy in Pulmonary Arterial Hypertension. JACC Cardiovasc Imaging. 2019 Nov;12(11 Pt 1):2103-2111. https://doi.org/10.1016/j.jcmg.2018.08.017

76. Farmakis IT, Demerouti E, Karyofyllis P, et al. Echocardiography in Pulmonary Arterial Hypertension: Is It Time to Reconsider Its Prognostic Utility? J Clin Med. 2021;10(13):2826. https://doi.org/10.3390/jcm10132826

77. Restrepo CS, Carswell AP. Aneurysms and pseudoaneurysms of the pulmonary vasculature. Semin Ultrasound CT MR. 2012;33(6):552-66. https://doi.org/10.1053/j.sult.2012.04.001

78. Berger T, Siepe M, Simon B, Beyersdorf F, Chen Z, Kondov S, Schlett CL, Bamberg F, Tarkhnishvili A, Chikvatia S, Czerny M, Rylski B, Kreibich M. Pulmonary artery diameter: means and normal limits-assessment by computed tomography angiography. Interact Cardiovasc Thorac Surg. 2022 Mar 31;34(4):637-644. https://doi.org/10.1093/icvts/ivab308

79. Reisenauer JS, Said SM, Schaff HV, et al. Outcome of Surgical Repair of Pulmonary Artery Aneurysms: A Single-Center Experience With 38 Patients. Ann Thorac Surg. 2017;104(5):1605-1610. https://doi.org/10.1016/j.athoracsur.2017.03.077

80. Duijnhouwer AL, Navarese EP, Van Dijk AP, et al. Aneurysm of the Pulmonary Artery, a Systematic Review and Critical Analysis of Current Literature. Congenit Heart Dis. 2016;11(2):102-109. https://doi.org/10.1111/chd.12316

81. Алеевская А.М., Выборов О.Н., Грамович В.В., Мартынюк Т.В. Особенности дифференциальной диагностики тромбоза лёгочной артерии у пациентов с лёгочной гипертензией (клиническое наблюдение). Пульмонология. 2019;29(5):620-624. https://doi.org/10.18093/0869-0189-2019-29-5-620-624

82. Mesquita SM, Castro CR, Ikari NM et al. Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension. Am J Med. 2004 Mar 15;116(6):369-74. https://doi.org/10.1016/j.amjmed.2003.11.015

83. Galiè N, Saia F, Palazzini M, et al. Left Main Coronary Artery Compression in Patients With Pulmonary Arterial Hypertension and Angina. J Am Coll Cardiol 2017; 69(23):2808-2817. https://doi.org/10.1016/j.jacc.2017.03.597

84. Kreibich M, Siepe M, Kroll J et al. Aneurysms of the pulmonary artery. Circulation 2015;131(3):310-316. https://doi.org/10.1161/circulationaha.114.012907

85. Tunariu N, Gibbs SJR, Win Z, et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med 2007; 48:680–684. https://doi.org/10.2967/jnumed.106.039438

86. Архипова О.А., Мартынюк Т.В., Самойленко Л.Е., Сергиенко В.Б., Чазова И.Е. Перфузионная сцинтиграфия лёгких у больных с лёгочной гипертензией различной этиологии. Евразийский Кардиологический Журнал. 2015;(4):20-25. https://doi.org/10.38109/2225-1685-2015-4-20-25

87. Хроническая тромбоэмболическая лёгочная гипертензия: Руководство для врачей/ Под ред. Т.В. Мартынюк, акад. РАН И.Е. Чазовой. – Москва: ООО «Медицинское информационное агентство», 2023. 416 с. ISBN 978-5-907098-61-9

88. Bajc M, Schümichen C, Grüning T, et al. EANM guideline for ventilation/perfusion single-photon emission computed tomography (SPECT) for diagnosis of pulmonary embolism and beyond. Eur J Nucl Med Mol Imaging. 2019; 46:2429–2451. https://doi.org/10.1007/s00259-019-04450-0

89. Masy M, Giordano J, Petyt G, et al. Dual-energy CT (DECT) lung perfusion in pulmonary hypertension: concordance rate with V/Q scintigraphy in diagnosing chronic thromboembolic pulmonary hypertension (CTEPH). Eur Radiol 2018; 28:5100–5110. https://doi.org/10.1007/s00330-018-5467-2

90. Dissaux, B, Le Floch PY, Robin P, et al. Pulmonary perfusion by iodine subtraction maps CT angiography in acute pulmonary embolism: comparison with pulmonary perfusion SPECT (PASEP trial). Eur Radiol 2020;30:4857–4864. https://doi.org/10.1007/s00330-020-06836-3

91. Johns CS, Swift AJ, Rajaram S, et al. Lung perfusion: MRI vs. SPECT for screening in suspected chronic thromboembolic pulmonary hypertension. J Magn Reson Imaging 2017; 46: 1693–1697. https://doi.org/10.1002/jmri.25714

92. Swift AJ, Dwivedi K, Johns C, et al. Diagnostic accuracy of CT pulmonary angiography in suspected pulmonary hypertension. Eur Radiol 2020;30:4918–4929. https://doi.org/10.1007/s00330-020-06846-1

93. Dong C, Zhou M, Liu D, et al. Diagnostic accuracy of computed tomography for chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis. PLoS One 2015;10:e0126985. https://doi.org/10.1371/journal.pone.0126985

94. Ende-Verhaar YM, Meijboom LJ, Kroft LJM, et al. Usefulness of standard computed tomography pulmonary angiography performed for acute pulmonary embolism for identification of chronic thromboembolic pulmonary hypertension: results of the InShape III study. J Heart Lung Transplant 2019;38:731–738. https://doi.org/10.1016/j.healun.2019.03.003

95. Guerin L, Couturaud F, Parent F, et al. Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of CTEPH after pulmonary embolism. Thromb Haemost 2014;112:598–605. https://doi.org/10.1160/th13-07-0538

96. Tamura M, Yamada Y, Kawakami T, et al. Diagnostic accuracy of lung subtraction iodine mapping CT for the evaluation of pulmonary perfusion in patients with chronic thromboembolic pulmonary hypertension: correlation with perfusion SPECT/CT. Int J Cardiol 2017;243:538–543. https://doi.org/10.1016/j.ijcard.2017.05.006

97. Веселова Т.Н., Демченкова А.Ю., Ен М.Ю., др. Оценка перфузии лёгких у больных хронической тромбоэмболической легочной гипертензией методами компьютерной томографии и сцинтиграфии. REJR. 2022;12(2):94-103. https://doi.org/10.21569/2222-7415-2022-12-2-94-103

98. Meyer GMB, Spilimbergo FB, Altmayer S, et al. Correction to: Multiparametric magnetic resonance imaging in the assessment of pulmonary hypertension: initial experience of a one-stop study. Lung 2018;196:497. https://doi.org/10.1007/s00408-018-0130-x

99. Swift AJ, Rajaram S, Condliffe R, et al. Diagnostic accuracy of cardiovascular magnetic resonance imaging of right ventricular morphology and function in the assessment of suspected pulmonary hypertension results from the ASPIRE registry. J Cardiovasc Magn Reson 2012; 14:40–50. https://doi.org/10.1186/1532-429x-14-40

100. Hatabu H, Ohno Y, Gefter WB, et al. Expanding applications of pulmonary MRI in the clinical evaluation of lung disorders: Fleischner society position paper. Radiology 2020; 297(2): 286-301. https://doi.org/10.1148/radiol.2020201138

101. Kiely DG, Levin D, Hassoun P, et al. EXPRESS: Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI).Pulm Circ. 2019; 9:2045894019841990. https://doi.org/10.1177/2045894019841990

102. Van de Veerdonk MC, Marcus JT, Bogaard HJ, et al. State of the art: advanced imaging of the right ventricle and pulmonary circulation in humans (2013 Grover Conference series). Pulm Circ 2014;4(2):158–168. https://doi.org/10.1086/675978

103. Vonk Noordegraaf A, Galie N. The role of the right ventricle in pulmonary arterial hypertension. Eur Respir Rev 2011; 20(122):243-253. https://doi.org/10.1183/09059180.00006511

104. Swift AJ, Wild JM, Nagle SK, et al. Quantitative magnetic resonance imaging of pulmonary hypertension a practical approach to the current state of the art. J Thorac Imag 2014; 29(2):68-79. https://doi.org/10.1097/rti.0000000000000079

105. Simpson CE, Damico RL, Kolb TM, Mathai SC, Khair RM, Sato T, Bourji K, Tedford RJ, Zimmerman SL, Hassoun PM. Ventricular mass as a prognostic imaging biomarker in incident pulmonary arterial hypertension. Eur Respir J. 2019 Apr 4;53(4):1802067. https://doi.org/10.1183/13993003.02067-2018

106. Johns CS, Kiely DG, Rajaram S, et al. Diagnosis of pulmonary hypertension with cardiac MRI: derivation and validation of regression models. Radiology. 2019;290:61–68. https://doi.org/10.1148/radiol.2018180603

107. Johns CS, Swift AJ, Hughes PJC, Ohno Y, Schiebler M, Wild JM. Pulmonary MR angiography and perfusion imaging-A review of methods and applications. Eur J Radiol. 2017 Jan;86:361-370. https://doi.org/10.1016/j.ejrad.2016.10.003

108. Rajaram S, Swift AJ, Capener D, et al. Diagnostic accuracy of contrast-enhanced MR angiography and unenhanced proton MR imaging compared with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension. Eur Radiol 2012; 22:310–317. https://doi.org/10.1007/s00330-011-2252-x

109. Alabed S, Shahin Y, Garg P, et al. Cardiac-MRI predicts clinical worsening and mortality in pulmonary arterial hypertension: a systematic review and meta-analysis. JACC Cardiovasc Imaging. 2020;29:S1936-878X(20)30731–2. https://doi.org/10.1016/j.jcmg.2020.08.013

110. Saunders LC, Johns CS, Stewart NJ, et al. Diagnostic and prognostic significance of cardiovascular magnetic resonance native myocardial T1 mapping in patients with pulmonary hypertension. J Cardiovasc Magn Reson. 2018;20:78. https://doi.org/10.1186/s12968-018-0501-8

111. Maschke SK, Schoenfeld CO, Kaireit TF, et al. MRI-derived regional biventricular function in patients with chronic thromboembolic pulmonary hypertension before and after pulmonary endarterectomy. Acad Radiol. 2018;25:1540–1547. https://doi.org/10.1016/j.acra.2018.04.002

112. Sieren MM, Berlin C, Oechtering TH, et al. Comparison of 4D flow MRI to 2D flow MRI in the pulmonary arteries in healthy volunteers and patients with pulmonary hypertension. PLoS One 2019; 14(10):e0224121. https://doi.org/10.1371/journal.pone.0224121

113. Saunders LC, Johns CS, Stewart NJ, Oram CJE, Capener DA, Puntmann VO, Elliot CA, Condliffe RC, Kiely DG, Graves MJ, Wild JM, Swift AJ. Diagnostic and prognostic significance of cardiovascular magnetic resonance native myocardial T1 mapping in patients with pulmonary hypertension. J Cardiovasc Magn Reson. 2018 Dec 3;20(1):78. https://doi.org/10.1186/s12968-018-0501-8

114. Naeije R. Hepatopulmonary syndrome and portopulmonary hypertension. SwissMed Wkly 2003; 133:163–169. https://doi.org/10.4414/smw.2003.10016

115. Rosenkranz S, Howard LS, Gomberg-Maitland M, Hoeper MM. Systemic consequences of pulmonary hypertension and right-sided heart failure. Circulation 2020; 141:678–693. https://doi.org/10.1161/circulationaha.116.022362

116. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013;369:809–818. https://doi.org/10.1056/NEJMoa1213917

117. Chu JW, Kao PN, Faul JL, Doyle RL. High prevalence of autoimmune thyroid disease in pulmonary arterial hypertension. Chest 2002; 122:1668-1673. https://doi.org/10.1378/chest.122.5.1668

118. Шмальц А.А. Динамика дистанции шестиминутной ходьбы как показатель эффективности специфических лёгочных вазодилататоров. Рациональная Фармакотерапия в Кардиологии. 2022;18(3):342-349. https://doi.org/10.20996/1819-6446-2022-06-09

119. Savarese G, Paolillo S, Costanzo P, et al. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials. J Am Coll Cardiol. 2012; 60(13): 1192–1201. https://doi.org/10.1016/j.jacc.2012.01.083

120. Таран И.Н., Валиева З.С., Мартынюк Т.В., др. Вклад спировелоэргометрии в диагностический алгоритм обследования больных с лёгочной артериальной гипертензией. Медицинский алфавит. Больница (кардиология). 2016;3:19-23.

121. Sherman AE, Saggar R. Cardiopulmonary Exercise Testing in Pulmonary Arterial Hypertension. Heart Fail Clin 2023;19(1):35-43. https://doi.org/10.1016/j.hfc.2022.08.015

122. Dumitrescu D, Nagel C, Kovacs G, et al. Cardiopulmonary exercise testing for detecting pulmonary arterial hypertension in systemic sclerosis. Heart 2017; 103:774–782. https://doi.org/10.1136/heartjnl-2016-309981

123. Torres-Castro R, Gimeno-Santos E, Vilaró J, et al. Effect of pulmonary hypertension on exercise tolerance in patients with COPD: a prognostic systematic review and meta-analysis.Eur Respir Review 2021; 30:200321. https://doi.org/10.1183/16000617.0321-2020

124. Hoeper MM, Lee SH, Voswinckel R, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 2006; 48: 2546-2552. https://doi.org/10.1016/j.jacc.2006.07.061

125. Kovacs G, Avian A, Olschewski A, Olschewski H. Zero reference level for right heart catheterisation. Eur Respir J 2013;42:1586–1594. https://doi.org/10.1183/09031936.00050713

126. Куропий Т.С., Данилов Н.М., Матчин Ю.Г. Сосудистые доступы к правым отделам сердца и легочным артериям: современное состояние вопроса. Кардиологический вестник 2022;17(3):23‑28. https://doi.org/10.17116/Cardiobulletin20221703123

127. Шмальц А.А., Мартынюк Т.В., Наконечников С.Н. Расчет гемодинамики методом Фика при лёгочной гипертензии, ассоциированной с врождёнными пороками сердца. Евразийский кардиологический журнал 2022;(2):96-102. https://doi.org/10.38109/2225-1685-2022-2-96-102

128. Opotowsky AR, Hess E, Maron BA, et al. Thermodilution vs estimated Fick cardiac output measurement in clinical practice: an analysis of mortality from the Veterans Affairs Clinical Assessment, Reporting, and Tracking (VA CART) Program and Vanderbilt University. JAMA Cardiol. 2017; 2:1090–1099. https://doi.org/10.1001/jamacardio.2017.2945

129. Viray MC, Bonno EL, Gabrielle ND, et al. Role of pulmonary artery wedge pressure saturation during right heart catheterization: a prospective study. Circ Heart Fail. 2020;13:e007981. https://doi.org/10.1161/circheartfailure.120.007981

130. Kubiak GM, Ciarka A, Biniecka M, et al. Right heart catheterization – background, physiological basics, and clinical implications. J Clin Med 2019;8:1331. https://doi.org/10.3390/jcm8091331

131. Kaemmerer H, Apitz C, Brockmeier K, et al. Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018;272S:79-88. https://doi.org/10.1016/j.ijcard.2018.08.078

132. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139(14):e698-e800. https://doi.org/10.1161/cir.0000000000000603

133. Xu Q, Yang Y, Geng J, et al. Clinical Study of Acute Vasoreactivity Testing in Patients with Chronic Thromboembolic Pulmonary Hypertension. Chin Med J (Engl) 2017;130(4):382-391. https://doi.org/10.4103/0366-6999.199829

134. Yang Y, Yu Y, Yuan P, et al. Sex differences of hemodynamics during acute vasoreactivity testing to predict the outcomes of chronic thromboembolic pulmonary hypertension. Eur Clin Respir J 2020; 14(7):611-621. https://doi.org/10.1111/crj.13173

135. Юрпольская Л.А., Шмальц А.А. Рентгенография, рентгеновская компьютерная и магнитно-резонансная томография при лёгочной гипертензии, ассоциированной с врождёнными пороками сердца. Детские болезни сердца и сосудов 2021; 18(2):83-93. https://dx.doi.org/10.24022/1810-0686-2021-18-2-83-93

136. Shimokawahara H, Ogawa A, Mizoguchi H, et al. Vessel stretching is a cause of lumen enlargement immediately after balloon pulmonary angioplasty: intravascular ultrasound analysis in patients with chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 2018;11:e006010. https://doi.org/10.1161/circinterventions.117.006010

137. Weatherald J, Montani D, Jevnikar M, et al. Screening for pulmonary arterial hypertension in systemic sclerosis. Eur Respir Rev. 2019;28:190023. https://doi.org/10.1183/16000617.0023-2019

138. Morrisroe K, Huq M, Stevens W, et al. Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: results from a large multicenter cohort study. BMC Pulm Med. 2016;16:134. https://doi.org/10.1186/s12890-016-0296-z

139. Волков А.В., Мартынюк Т.В. Лёгочная артериальная гипертензия при системных заболеваниях соединительной ткани: современное состояние проблемы. Научно-практическая ревматология 2018;56(4):474−485. https://doi.org/10.14412/1995-4484-2018-474-485

140. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005;52:3792–3800. https://doi.org/10.1002/art.21433

141. Humbert M, Yaici A, de Groote P, et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum. 2011;63:3522–3530. https://doi.org/10.1002/art.30541

142. Coghlan JG, Denton CP, Grunig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis:the DETECT study. Ann Rheum Dis. 2014;73:1340–134. https://doi.org/10.1136/annrheumdis-2013-203301

143. Morrell NW, Aldred MA, Chung WK, et al. Genetics and genomics of pulmonary arterial hypertension. Eur Respir J 2019;53:1801899. https://doi.org/10.1183/13993003.01899-2018

144. Kiely DG, Lawrie A, Humbert M. Screening strategies for pulmonary arterial hypertension. Eur Heart J Suppl. 2019; 21:K9–K20. https://doi.org/10.1093/eurheartj/suz204

145. Boon G, Ende-Verhaar YM, Bavalia R, et al. Non-invasive early exclusion of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: the InShape II study. Thorax 2021;76:1002–1009. https://doi.org/10.1136/thoraxjnl-2020-216324

146. Kylhammar D, Kjellstrom B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J 2018;39:4175–4181. https://doi.org/10.1093/eurheartj/ehx257

147. 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; 50:1700740. https://doi.org/10.1183/13993003.00740-2017

148. Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J 2017; 50:1700889. https://doi.org/10.1183/13993003.00889-2017

149. Benza RL, Kanwar MK, Raina A, et al. Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension. Chest. 2021;159: 337–346. https://doi.org/10.1016/j.chest.2020.08.2069

150. Zelniker TA, Huscher D, Vonk-Noordegraaf A, et al. The 6MWT as a prognostic tool in pulmonary arterial hypertension: results from the COMPERA registry. Clin Res Cardiol. 2018;107(6):460-70. https://doi.org/10.1007/s00392-018-1207-5

151. Provencher S, Chemla D, Herve P, et al. Heart rate responses during the 6-minute walk test in pulmonary arterial hypertension. Eur Respir J. 2006;27:114–120. https://doi.org/10.1183/09031936.06.00042705

152. Khirfan G, Naal T, Abuhalimeh B, et al. Hypoxemia in patients with idiopathic or heritable pulmonary arterial hypertension. PLoS One. 2018;13: e0191869. https://doi.org/10.1371/journal.pone.0191869

153. Sitbon O, Chin KM, Channick RN, et al. Risk assessment in pulmonary arterial hypertension: insights from the GRIPHON study. J Heart Lung Transpl. 2020;39:300–309. https://doi.org/10.1016/j.healun.2019.12.013

154. Nickel N, Golpon H, Greer M, et. al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2012; 39:589–596. https://doi.org/10.1183/09031936.00092311

155. Farber HW, Miller DP, McGoon MD, et al. Predicting outcomes in pulmonary arterial hypertension based on the 6-minute walk distance. J Heart Lung Transplant. 2015; 34(3):362–368. https://doi.org/10.1016/j.healun.2014.08.020

156. Groepenhoff H, Vonk-Noordegraaf A, van de Veerdonk MC, et al. Prognostic relevance of changes in exercise test variables in pulmonary arterial hypertension. PLoS One. 2013; 8(9):e72013. https://doi.org/10.1371/journal.pone.0072013

157. Schiebler ML, Bhalla S, Runo J, et al. Magnetic resonance and computed tomography imaging of the structural and functional changes of pulmonary arterial hypertension. J Thorac Imaging. 2013; 28(3):178–193. https://doi.org/10.1097/rti.0b013e31828d5c48

158. de Siqueira MEM, Pozo E, Fernandes VR, et al. Characterization and clinical significance of right ventricular mechanics in pulmonary hypertension evaluated with cardiovascular magnetic resonance feature tracking. J Cardiovasc Magn Reson. 2016;18(1):39. https://doi.org/10.1186/s12968-016-0258-x

159. Leuchte HH, El Nounou M, Tuerpe JC, et al. N-terminal pro-brain natriuretic peptide and renal insufficiency as predictors of mortality in pulmonary hypertension. Chest 2007; 131(2):402–409. https://doi.org/10.1378/chest.06-1758

160. Mereles D, Ehlken N, Kreuscher S, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation 2006; 114:1482-1489. https://doi.org/10.1161/circulationaha.106.618397

161. Grunig E, MacKenzie A, Peacock AJ, et al. Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial. Eur Heart J 2021;42:2284–2295. https://doi.org/10.1093/eurheartj/ehaa696

162. Morris N.R., Kermeen F.D., Holland A.E. Exercise-based rehabilitation programs for pulmonary hypertension. Cochrane Database of Systematic Reviews. 2017;1(1):CD011285. https://doi.org/10.1002/14651858.cd011285.pub2

163. Chia KSW., Wong PKK., Faux SG. et al. The benefit of exercise training in pulmonary hypertension: a clinical review. Intern Med J 2017; 47(4):361–369. https://doi.org/10.1111/imj.13159

164. Halvorsen S, Mehilli J, Cassese S, et al. 2022 ESC Guidelines on cardiovascular assessment and management of patients undergoing non-cardiac surgery. Eur Heart J. 2022;43(39):3826-3924. https://doi.org/10.1093/eurheartj/ehac270

165. Диагностика и лечение сердечно-сосудистых заболеваний при беременности 2018. Национальные рекомендации. Российский кардиологический журнал 2018, 3(155):91–134. https://doi.org/10.15829/1560-4071-2018-3-91-134

166. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, et al. 2018 ESC guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J 2018; 39: 3165–3241. https://doi.org/10.1093/eurheartj/ehy340

167. Hemnes AR, Kiely DG, Cockrill BA, et al. Statement on pregnancy in pulmonary hypertension from the Pulmonary Vascular Research Institute. Pulm Circ. 2015; 5:435-465. https://doi.org/10.1086/682230

168. Duarte AG, Thomas S, Safdar Z, et al. Management of pulmonary arterial hypertension during pregnancy: a retrospective, multicenter experience. Chest 2013;143:1330–1336. https://doi.org/10.1378/chest.12-0528

169. Hill W., Holy R., Traiger G. EXPRESS: Intimacy, Contraception, and Pregnancy Prevention in Patients with Pulmonary Arterial Hypertension: Are We Counseling Our Patients? Pulm Circ. 2018;10(4):2045894018785259. https://doi.org/10.1177/2045894018785259

170. Gemzell-Danielsson K, Rabe T, Cheng L. Emergency contraception. Gynecol Endocrinol 2013; 29:1–14. https://doi.org/10.3109/09513590.2013.774591

171. Meyer S, McLaughlin VV, Seyfarth HJ, et al. Outcomes of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey. Eur Respir J. 2013;41:1302–1307. https://doi.org/10.1183/09031936.00089212

172. Thangamathesvaran L, Armenia SJ, Merchant AM. The effect of pulmonary hypertension on inpatient outcomes of laparoscopic procedures. Updates Surg. 2018 Dec;70(4):521-528. https://doi.org/10.1007/s13304-018-0556-y

173. Чазова И.Е, Мартынюк Т.В, Валиева З.С, др. Оценка бремени хронической тромбоэмболической лёгочной гипертензии в Российской Федерации. Терапевтический архив. 2018; 9: 101-109. https://doi.org/10.26442/terarkh2018909101-109

174. Wang P, Hu L, Yin Y, et al. Can anticoagulants improve the survival rate for patients with idiopathic pulmonary arterial hypertension? A systematic review and meta-analysis. Thromb Res. 2020;196:251–256. https://doi.org/10.1016/j.thromres.2020.08.024

175. Olsson KM, Delcroix M, Ghofrani HA, et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation 2014; 129:57–65. https://doi.org/10.1161/circulationaha.113.004526

176. Грацианская С.Е., Мартынюк Т.В., Наконечников С.Н., Чазова И.Е. Проблемные аспекты применения оральных антикоагулянтов у пациентов с легочной артериальной гипертензией различного генеза. Евразийский Кардиологический Журнал. 2017;(4):122-132. https://doi.org/10.38109/2225-1685-2017-4-122-132

177. Чазова И.Е., Мартынюк Т.В., Филиппов Е.В., соавт. Клинические рекомендации по диагностике и лечению хронической тромбоэмболической лёгочной гипертензии. (2 ЧАСТЬ). Терапевтический архив 2016; 88(10):63-73. https://doi.org/10.17116/terarkh201688663-73

178. Mancuso L, Scordato F, Pieri M, et al. Management of portopulmonary hypertension: new perspectives. World J Gastroenterol. 2013;19:8252–8257. https://doi.org/10.3748/wjg.v19.i45.8252

179. Condliffe R, Kiely DG. Critical care management of pulmonary hypertension. BJA Education 2017; 17(7):228–234. https://doi.org/10.1093/bjaed/mkw074

180. Stickel S, Gin-Sing W, Wagenaar M, Gibbs JSR. The practical management of fluid retention in adults with right heart failure due to pulmonary arterial hypertension. Eur Heart J Suppl. 2019;21:K46–K53. https://doi.org/10.1093/eurheartj/suz207

181. Rich S, Seidlitz M, Dodin E, et al. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest 1998;114:787-792. https://doi.org/10.1378/chest.114.3.787

182. Tongers J, Schwerdtfeger B, Klein G, et al. Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J 2007; 153:127-132. https://doi.org/10.1016/j.ahj.2006.09.008

183. Olsson KM, Nickel NP, Tongers J, Hoeper MM. Atrial flutter and fibrillation in patients with pulmonary hypertension. Int J Cardiol 2013; 167:2300–2305. https://doi.org/10.1016/j.ijcard.2012.06.024

184. Hoeper MM, Benza RL, Corris P, et al. Intensive care, right ventricular support and lung transplantation in patients with pulmonary hypertension. Eur Respir J 2019; 53:1801906. https://doi.org/10.1183/13993003.01906-2018

185. Jentzer JC, Mathier, MA. Pulmonary Hypertension in the Intensive Care Unit J Intensive Care Med 2016; 31(6):369–385. https://doi.org/10.1177/0885066615583652

186. Sztrymf B, Souza R, Bertoletti I, et al. Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension. Eur Respir J 2010; 35:1286–1293. https://doi.org/10.1183/09031936.00070209

187. Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327:76-81. https://doi.org/10.1056/nejm199207093270203

188. Clozel M, Maresta A, Humbert M. Endothelin receptor antagonists. Handb Exp Pharmacol. 2013;218:199–227. https://doi.org/10.1007/978-3-642-38664-0_9

189. Galiè N, Olschewski H, Oudiz RJ, et al. 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; 117:3010–3019. https://doi.org/10.1161/circulationaha.107.742510

190. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346:896–903. https://doi.org/10.1056/nejmoa012212

191. Humbert M, Segal ES, Kiely DG, et al. Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J. 2007;30:338–344. https://doi.org/10.1183/09031936.00138706

192. Sitbon J, Bosch J, Cottreel E, et al. Macitentan for the treatment of portopulmonary hypertension (PORTICO): a multicentre, randomised, double-blind, placebo-controlled, phase 4 trial. Lancet Respir Med 2019; 7(7):594-604. https://doi.org/10.1016/s2213-2600(19)30091-8

193. Galie N, Ghofrani HA, Torbicki A, et al, the Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353:2148–2157. https://doi.org/10.1056/nejmoa050010

194. Michelakis ED, Tymchak W, Noga M, et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation 2003;108:2066-2069. https://doi.org/10.1161/01.cir.0000099502.17776.c2

195. Galiè N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009;119:2894–2903. https://doi.org/10.1161/circulationaha.108.839274

196. Lichtblau, M., Harzheim, D., Ehlken, N., et al. Safety and long-term efficacy of transition from sildenafil to tadalafil due to side effects in patients with pulmonary arterial hypertension. Lung 193; 1:105–112. https://doi.org/10.1007/s00408-014-9657-7

197. Garraffo R, Lavrut T, Ferrando S, et al. Effect of tipranavir/ritonavir combination on the pharmacokinetics of tadalafil in healthy volunteers. J Clin Pharmacol. 2011;51:1071–1078. https://doi.org/10.1177/0091270010379808

198. Schermuly RT, Janssen W, Weissmann N, et al. Riociguat for the treatment of pulmonary hypertension. Expert Opin Investig Drugs. 2011;20:567–576. https://doi.org/10.1517/13543784.2011.565048

199. Ghofrani H-A, Galiè N, Grimminger F, et al. Riociguat for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med 2013;369:330-40. https://doi.org/10.1056/nejmoa1209655

200. 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):319-329. https://doi.org/10.1056/nejmoa1209657

201. Ghofrani H-A, Grimminger F, Grünig E, et al. Predictors of long-term outcomes in patients treated with riociguat for pulmonary arterial hypertension: data from the PATENT-2 open-label, randomized, long-term extension trial. Lancet Respir Med. 2016; 4:361–371. https://doi.org/10.1016/s2213-2600(16)30019-4

202. Simonneau G, D’Armini AM, Ghofrani HA et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J 2015;45(5):1293-302. https://doi.org/10.1183/09031936.00087114

203. Olschewski H. Inhaled iloprost for the treatment of pulmonary hypertension. Eur Respir Rev. 2009; 18(111):29–34. https://doi.org/10.1183/09059180.00011111

204. 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;174:1257–1263. https://doi.org/10.1164/rccm.200603-358oc

205. Hoeper MM, Leuchte H, Halank M, et al. Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2006;28:691–694. https://doi.org/10.1183/09031936.06.00057906

206. Мартынюк Т.В. Оценка приверженности пациентов с лёгочной гипертензией лечению ингаляционным илопростом (Вентавис): итоги проспективного многоцентрового неинтервенционного исследования IVENT. Системные гипертензии. 2019;16(2):12-27. https://doi.org/10.26442/2075082X.2019.2.190352

207. Sitbon O, Channick R, Chin KM, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373:2522–2533. https://doi.org/10.1056/nejmoa1503184

208. Резухина Е.А., Родненков О.В., Мартынюк Т.В. Портрет типичного пациента с лёгочной артериальной гипертензией для назначения агониста простациклиновых рецепторов селексипага. Евразийский Кардиологический Журнал 2023;(1):94-99. https://doi.org/10.38109/2225-1685-2023-1-94-99

209. Boucly A, Savale, L, Jaïs, X, et al. Association between Initial Treatment Strategy and Long-Term Survival in Pulmonary Arterial Hypertension. Am J of Respir and Crit Care Med. 2021; 204(7):842–854. https://doi.org/10.1164/rccm.202009-3698oc

210. 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. https://doi.org/10.1016/j.ijcard.2018.08.082

211. Hoeper MM, McLaughlin VV, Barbera JA, et al. Initial combination therapy with ambrisentan and tadalafil and mortality in patients with pulmonary arterial hypertension: a secondary analysis of the results from the randomized, controlled AMBITION study. Lancet Respir Med 2016;4:894–901. https://doi.org/10.1016/S2213-2600(16)30307-1

212. McLaughlin VV, Vachiery, JL, Oudiz, RJ, et al. (AMBITION Study Group). Patients with pulmonary arterial hypertension with and without cardiovascular risk factors: Results from the AMBITION trial. J of Heart and Lung transplantation: the official publication of the International Society for Heart Transplantation. 2019; 38(12):1286–1295. https://doi.org/10.1016/j.healun.2019.09.010

213. Lang I M, Palazzini, M. The burden of comorbidities in pulmonary arterial hypertension. Eur Heart J Suppl. 2019; 21(Suppl K):K21–K28. https://doi.org/10.1093/eurheartj/suz205

214. Шмальц А.А., Горбачевский С.В. Возможности и ограничения для начальной комбинированной специфической терапии лёгочной артериальной гипертензии в Российской Федерации. Терапевтический архив 2020; 92(12):80-85. https://doi.org/10.26442/00403660.2020.12.000840

215. Ляпина И.Н., Зверева Т.Н., Мартынюк Т.В. Возможности комбинированной терапии в лечении пациентов с впервые установленным диагнозом лёгочной артериальной гипертензии промежуточного риска. Consilium Medicum 2022; 24(10):688-695. https://doi.org/10.26442/20751753.2022.10.201875

216. Galiè N, Barbera JA, Frost AE, et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015;373:834–844. https://doi.org/10.1056/nejmoa1413687

217. Hassoun PM, Zamanian RT, Damico R, et al. Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2015;192:1102–1110. https://doi.org/10.1164/rccm.201507-1398oc

218. Sitbon O, Cottin V, Canuet M, et al. Initial combination therapy of macitentan and tadalafil in pulmonary arterial hypertension. Eur Respir J;56(3):2000673. https://doi.org/10.1183/13993003.00673-2020

219. Macitentan/Tadalafil Fixed-Dose Combination in Pulmonary Arterial Hypertension - A DUE. Presented by Dr. Kelly Chin at the American College of Cardiology Annual Scientific Session (ACC.23/WCC), New Orleans, LA, March 6, 2023. https://www.acc.org/Latest-in-Cardiology/Clinical-Trials/2023/03/01/23/47/a-due

220. 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;78:1393–1403. https://doi.org/10.1016/j.jacc.2021.07.057

221. Thakrar MV, Weatherald JC, Varughese RA, et al. Initial Combination Therapy With Riociguat and Ambrisentan in Pulmonary Arterial Hypertension: A Prospective Open-label Study. J Heart Lung Transplant. 2018;37(4S):S53-S54. https://doi.org/10.1016/j.healun.2018.01.115

222. Satoshi A, Yoshihiro D, Kaori I, et al. Effects of Dual Initial Combination Therapy With Macitentan Plus Riociguat or Macitentan Plus Selexipag on Hemodynamics in Patients With Pulmonary Arterial Hypertension (SETOUCHI-PH Study) – Protocol of a Multicenter Randomized Control Trial. Circ Rep. 2021;3(2):105-109. https://doi.org/10.1253/circrep.cr-20-0133

223. Gaine S, Sitbon O, Channick RN, et al. Relationship Between Time From Diagnosis and Morbidity/Mortality in Pulmonary Arterial Hypertension: Results From the Phase III GRIPHON Study. Chest 2021; 160(1):277–286. https://doi.org/10.1016/j.chest.2021.01.066

224. 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):405-413. https://doi.org/10.1183/13993003.02044-2014

225. Galiè, N. Gaine S, Channick R, et al. Long-Term Survival, Safety and Tolerability with Selexipag in Patients with Pulmonary Arterial Hypertension: Results from GRIPHON and its Open-Label Extension. Advances in therapy 2022; 39(1):796-810. https://doi.org/10.1007/s12325-021-01898-1

226. Hoeper MM, Simonneau G, Corris PA, et al. RESPITE: switching to riociguat in pulmonary arterial hypertension patients with inadequate response to phosphodiesterase-5 inhibitors. Eur Respir J 2017;50(3):1602425. https://doi.org/10.1183/13993003.02425-2016

227. 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):745-753. https://doi.org/10.1007/s00408-018-0160-4

228. Мартынюк Т.В., Шмальц А.А., Горбачевский С.В., Чазова И.Е. Оптимизация специфической терапии лёгочной гипертензии: возможности риоцигуата. Терапевтический архив 2021; 93(9):1117–1124. https://doi.org/10.26442/00403660.2021.09.201014

229. Мартынюк Т.В., Наконечников С.Н., Чазова И.Е. Новые горизонты применения антагониста рецепторов эндотелина второго поколения мацитентана у пациентов с лёгочной гипертензией. Терапевтический архив 2018; 2018:72-80. https://ter-arkhiv.ru/0040-3660/article/view/32480

230. Hoeper, MM, Al-Hiti, H, Benza, RL, et al. Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial. The Lancet Respir Med 2021; 9(6)573–584. https://doi.org/10.1016/s2213-2600(20)30532-4

231. Чазова И.Е., Мартынюк Т.В. от имени авторов. Резолюция экспертного совета по проблеме лёгочной артериальной гипертензии «Меняя парадигму лечения пациентов с ЛАГ». Терапевтический архив 2019;91(3):114-116. https://doi.org/10.26442/00403660.2019.03.000185

232. Li J, Yang ZY, Wang S, et al. Efficacy and safety of switching from bosentan or ambrisentan to macitentan in pulmonary arterial hypertension: A systematic review and meta-analysis. Front Cardiovasc Med 2022;9:977110. https://doi.org/10.3389/fcvm.2022.977110

233. Sandoval J, Gomez-Arroyo J, Gaspar J, Pulido-Zamudio T. Interventional and surgical therapeutic strategies for pulmonary arterial hypertension: Beyond palliative treatments. J Cardiol. 2015; 66(4):304-314. https://doi.org/10.1016/j.jjcc.2015.02.001

234. Gorbachevsky SV, Shmalts AA, Dadabaev GM, et al. Outcomes of Atrioseptostomy with Stenting in Patients with Pulmonary Arterial Hypertension from a Large Single-Institution Cohort. Diagnostics (Basel). 2020;10(9):725. https://doi.org/10.3390/diagnostics10090725

235. Горбачевский С.В., Пурсанов М.Г., Шмальц А.А., др. Результаты атриосептостомии со стентированием у больных с идиопатической и схожими формами лёгочной артериальной гипертензии. Грудная и сердечно-сосудистая хирургия. 2019; 61(2):100-113. https://dx.doi.org/10.24022/0236-2791-2019-61-2-100-113

236. Шмальц А.А., Нишонов Н.А. Атриосептостомия у больных с лёгочной гипертензией. Грудная и сердечно-сосудистая хирургия. 2015;57(5):18-25.

237. Khan MS, Memon MM, Amin E, et al. Use of balloon atrial septostomy in patients with advanced pulmonary arterial hypertension: a systematic review and meta-analysis. Chest. 2019; 156:53–63. https://doi.org/10.1016/j.chest.2019.03.003

238. Bartolome SD., Torres F. Severe pulmonary arterial hypertension: stratification of medical therapies, mechanical support, and lung transplantation. Heart Fail Rev. 2016; 21(3):347–356. https://doi.org/10.1007/s10741-016-9562-7

239. Hoeper MM, Benza RL, Corris P, et al. Intensive care, right ventricular support and lung transplantation in patients with pulmonary hypertension. Eur Respir J 2019; 53:1801906. https://doi.org/10.1183/13993003.01906-2018

240. Шмальц А.А., Горбачевский С.В., Мартынюк Т.В., Наконечников С.Н. Тест на вазореактивность при лёгочной гипертензии, ассоциированной с врождёнными пороками сердца. Евразийский кардиологический журнал 2022; 3:58-64. https://doi.org/10.38109/2225-1685-2022-3-58-64

241. Горбачевский С.В., Шмальц А.А., Плотникова Л.Р. Лёгочная гипертензия у детей с врождёнными пороками сердца. М.: ООО «Кедр»; 2018. ISBN 978-5-91487-115-1

242. Барышникова И.Ю., Шмальц А.А. Диагностика лёгочной гипертензии, ассоциированной с врождёнными пороками сердца. Часть 2. Эхокардиография. Российский вестник перинатологии и педиатрии 2021;66:(6):23–32. https://doi.org/10.21508/1027-4065-2021-66-6-23-32

243. Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J 2021;42(6):563-645. https://doi.org/10.1093/eurheartj/ehaa554

244. Соболев А.В., Шмальц А.А. Эндоваскулярная диагностика лёгочной гипертензии, ассоциированной с врождёнными пороками сердца. Часть 1. Катетеризация сердца и ангиокардиография. Эндоваскулярная хирургия 2021; 8(3):263–71. https://doi.org/https://doi.org/10.24183/2409-4080-2021-8-3-263-271

245. Горбачевский С.В., Шмальц А.А. Первичное обследование. В кн.: Горбачевский С.В., Шмальц А.А. (ред.) Диагностика лёгочной гипертензии при врождённых пороках сердца. М.: ФГБУ «НМИЦ ССХ им. А.Н.Бакулева» МЗ РФ; 2023:20-34. ISBN: 978-5-7982-0450-2

246. Барышникова И.Ю., Шмальц А.А. Эхокардиография. В кн.: Горбачевский С.В., Шмальц А.А. (ред.) Диагностика лёгочной гипертензии при врождённых пороках сердца. М.: ФГБУ «НМИЦ ССХ им. А.Н.Бакулева» МЗ РФ; 2023:35-52. ISBN: 978-5-7982-0450-2

247. Юрпольская Л.А., Шмальц А.А. Рентгеновская компьютерная и магнитно-резонансная томография. В кн.: Горбачевский С.В., Шмальц А.А. (ред.) Диагностика лёгочной гипертензии при врождённых пороках сердца. М.: ФГБУ «НМИЦ ССХ им. А.Н.Бакулева» МЗ РФ; 2023:53-67. ISBN: 978-5-7982-0450-2

248. Соболев А.В., Шмальц А.А. Катетeризация сердца и ангиокардиография. В кн.: Горбачевский С.В., Шмальц А.А. (ред.) Диагностика лёгочной гипертензии при врождённых пороках сердца. М.: ФГБУ «НМИЦ ССХ им. А.Н.Бакулева» МЗ РФ; 2023:68-83. ISBN: 978-5-7982-0450-2

249. Глушко Л.А., Шмальц А.А. Оценка состояния кардиреспираторной системы. В кн.: Горбачевский С.В., Шмальц А.А. (ред.) Диагностика лёгочной гипертензии при врождённых пороках сердца. М.: ФГБУ «НМИЦ ССХ им. А.Н.Бакулева» МЗ РФ; 2023:109-124. ISBN: 978-5-7982-0450-2

250. Шмальц А.А., Горбачевский С.В. Тест на вазореактивность. В кн.: Горбачевский С.В., Шмальц А.А. (ред.) Диагностика лёгочной гипертензии при врождённых пороках сердца. М.: ФГБУ «НМИЦ ССХ им. А.Н.Бакулева» МЗ РФ; 2023:99-108. ISBN: 978-5-7982-0450-2

251. Arvind B, Relan J, Kothari SS. “Treat and repair” strategy for shunt lesions: a critical review. Pulm Circ 2020;10:2045894020917885. https://doi.org/10.1177/2045894020917885

252. Шмальц А.А., Белкина М.В., Горбачевский С.В. Специфические легочные вазодилататоры после операции Фонтена. Детские болезни сердца и сосудов 2017;14(1):16-24. https://dx.doi.org/10.24022/1810-0686-2017-14-1-16-24

253. Galiè N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006; 114:48–54. https://doi.org/10.1161/circulationaha.106.630715

254. Шмальц А.А., Горбачевский С.В. Доказательная база специфических лёгочных вазодилататоров у взрослых с врождёнными пороками сердца. Терапевтический архив. 2021;93(9):1106-1116. https://doi.org/10.26442/00403660.2021.09.201022

255. Rubio-Rivas M, Royo C, Simeón CP, et al.. Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum. 2014;44(2):208-19. https://doi.org/10.1016/j.semarthrit.2014.05.010

256. McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev. 2012;21(123):8-18. https://doi.org/10.1183/09059180.00008211

257. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023-30. https://doi.org/10.1164/rccm.200510-1668oc

258. Гусева, Н.Г. Системная склеродермия – мультидисциплинарная проблема. Научно-практическая ревматология 2011;49(2):10-14. https://doi.org/10.14412/1995-4484-2011-596

259. Волков А.В., Мартынюк Т.В., Юдкина Н.Н. и др. Выживаемость пациентов с лёгочной артериальной гипертонией, ассоциированной с системной склеродермией. Терапевтический архив 2012;84(5):24-28. https://ter-arkhiv.ru/0040-3660/article/view/31020

260. Attanasio U, Cuomo A, Pirozzi F, et al. Pulmonary Hypertension Phenotypes in Systemic Sclerosis: The Right Diagnosis for the Right Treatment. Int J Mol Sci. 2020;21(12):4430. https://doi.org/10.3390/ijms21124430

261. Николаева Е.В., Корсакова Ю.О., Курмуков И.А. и др. Возможности эхокардиографического определения давления в лёгочной артерии у пациентов с системными заболеваниями соединительной ткани: данные ревматологического экспертного центра. Научно-практическая ревматология 2015;53(1):51-57. https://doi.org/10.14412/1995-4484-2015-51-57

262. Юдкина Н.Н., Николаева Е.В., Мартынюк Т.В. и др. Клинический субтип системной склеродермии, ассоциированный с лёгочной артериальной гипертензией. Кардиологический вестник. 2017;12(4):66-75.

263. Simpson CE, Damico RL, Hummers L, et al. Serum uric acid as a marker of disease risk, severity, and survival in systemic sclerosis-related pulmonary arterial hypertension. Pulm Circ. 2019;9(3):2045894019859477. https://doi.org/10.1177/2045894019859477

264. Santos-Gomes J, Gandra I, Adão R, et al. An Overview of Circulating Pulmonary Arterial Hypertension Biomarkers. Front Cardiovasc Med 2022;9:924873. https://doi.org/10.3389/fcvm.2022.924873

265. Hao Y, Thakkar V, Stevens W, et al. A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis. Arthritis Res Ther. 2015;17(1):7. https://doi.org/10.1186/s13075-015-0517-5

266. Savale L, Guimas M, Ebstein N, Fertin M, et al. Portopulmonary hypertension in the current era of pulmonary hypertension management. J Hepatol 2020;73:130–139. https://doi.org/10.1016/j.jhep.2020.02.021

267. Ryom L, Cotter A, De Miguel R, et al. 2019 update of the European AIDS Clinical Society Guidelines for treatment of people living with HIV version 10.0. HIV Med. 2020;21:617–624. https://doi.org/10.1111/hiv.12878

268. Garry JD, Kolaitis NA, Kronmal R, et al. Pulmonary Hypertension Association Registry Investigators. Anticoagulation in pulmonary arterial hypertension - association with mortality, healthcare utilization, and quality of life: The Pulmonary Hypertension Association Registry (PHAR). J Heart Lung Transplant. 2022;41(12):1808-1818. https://doi.org/10.1016/j.healun.2022.08.019

269. Opravil M, Sereni D. Natural history of HIV-associated pulmonary arterial hypertension: trends in the HAART era. AIDS (London, England) 2008;22:S35–S40. https://doi.org/10.1097/01.aids.0000327514.60879.47

270. Mularek-Kubzdela T, Ciurzyński M, Kowal Bielecka O, et al. An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease. Kardiol Pol. 2021;79(7-8):917-929. https://doi.org/10.33963/kp.a2021.0055

271. Конева О.А., Овсянникова О.Б., М. Н. Старовойтова и др. Изолированное снижение диффузионной способности лёгких при системной склеродермии без лёгочной артериальной гипертензии: длительное проспективное наблюдение. Пульмонология. 2016;26(6):708-714. https://doi.org/10.18093/0869-0189-2016-26-6-708-714

272. Xanthouli P. Improved Survival for Patients with Systemic Sclerosis-associated Pulmonary Arterial Hypertension: For Real? Am J Respir Crit Care Med. 2023;207(3):238-240. https://doi.org/10.1164/rccm.202210-2006ed

273. Lazaro SM, Quezada Loaiza CA, Rodriguez Padial, et al. Portopulmonary hypertension: prognosis and management in the current treatment era – results from the REHAP registry. Intern Med. 2021;51:355–365. https://doi.org/10.1111/imj.14751

274. Krowka MJ, Miller DP, Barst RJ, et al. Portopulmonary hypertension: a report from the US-based REVEAL Registry. Chest 2012;141:906–915. https://doi.org/10.1378/chest.11-0160

275. Sitbon O, Gressin V, Speich R, et al. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;170:1212–1217. https://doi.org/10.1164/rccm.200404-445oc

276. Certain MC, Chaumais MC, Jais X, et al. Characteristics and long-term outcomes of pulmonary venoocclusive disease induced by mitomycin C. Ches. 2021;159:1197–1207. https://doi.org/10.1016/j.chest.2020.09.238

277. Montani D, Girerd B, Jais X, et al. Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study. Lancet Respir Med 2017;5:125–134. https://doi.org/10.1016/s2213-2600(16)30438-6

278. Меркулов Е.В., Сапельников О.В., Власова Э.Е., др. Осложнения катетерной абляции у пациента с фибрилляцией предсердий: стеноз всех лёгочных вен. Особенности эндоваскулярного лечения. Кардиология 2019;59(5):92-96. https://doi.org/10.18087/cardio.2019.5.10260

279. Assad TR, Hemnes AR, Larkin EK et al. Clinical and Biological Insights Into Combined Post- and Pre-Capillary Pulmonary Hypertension. J Am Coll Cardiol 2016; 68(23):2525-2536. https://doi.org/10.1016/j.jacc.2016.09.942

280. Halpern SD, Taichman DB. Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure. Chest 2009;136(1):37-43. https://doi.org/10.1378/chest.08-2784

281. McDonagh TA, Metra M, Adamo M, et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J 2021; 42:3599–3726. https://doi.org/10.1093/eurheartj/ehab368

282. Bermejo J, Gonzalez-Mansilla A, Mombiela T, et al. Persistent pulmonary hypertension in corrected valvular heart disease: hemodynamic insights and long-term survival. J Am Heart Assoc. 2021;10: e019949. https://doi.org/10.1161/jaha.120.019949

283. Kovacs G, Herve P, Barbera JA, et al. An official European Respiratory Society statement: pulmonary haemodynamics during exercise. Eur Respir J. 2017;50:1700578. https://doi.org/10.1183/13993003.00578-2017

284. D’Alto M, Romeo E, Argiento P, et al. Clinical relevance of fluid challenge in patients evaluated for pulmonary hypertension. Chest. 2017;151:119–126. https://doi.org/10.1016/j.chest.2016.08.1439

285. Koller B, Steringer-Mascherbauer R, Ebner CH, et al. Pilot study of endothelin receptor blockade in heart failure with diastolic dysfunction and pulmonary hypertension (BADDHY-trial). Heart Lung Circ 2017;26:433–441. https://doi.org/10.1016/j.hlc.2016.09.004

286. Vachiery JL, Delcroix M, Al-Hiti H, et al. Macitentan in pulmonary hypertension due to left ventricular dysfunction. Eur Respir J 2018;51:1701886. https://doi.org/10.1183/13993003.01886-2017

287. Bermejo J, Yotti R, Garcia-Orta R, et al. Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension: a multicenter, double-blind, randomized clinical trial. Eur Heart J. 2018; 39:1255–1264. https://doi.org/10.1093/eurheartj/ehx700

288. Kaluski E, Cotter G, Leitman M, et al. Clinical and hemodynamic effects of bosentan dose optimization in symptomatic heart failure patients with severe systolic dysfunction, associated with secondary pulmonaryhypertension–a multi-center randomized study. Cardiology. 2008;109:273–280. https://doi.org/10.1159/000107791

289. Cao JY, Wales KM, Cordina R, Lau EMT, Celermajer DS. Pulmonary vasodilator therapies are of no benefit in pulmonary hypertension due to left heart disease: A meta-analysis. Int J Cardiol 2018;273:213–220. https://doi.org/10.1016/j.ijcard.2018.09.043

290. Lilly LS. Pathophysiology of Heart Disease: An Introduction to Cardiovascular Medicine (p. 249). Wolters Kluwer Health, 7 th. ed., 2021; Kindle Edition. ISBN-13: 978-1975120597

291. Ляпина И.Н., Теплова Ю.Е., Мартынюк Т.В. Фенотип пациентов с лёгочной гипертензией вследствие патологии левых отделов сердца: особенности патофизиологии и дифференциальной диагностики. Комплексные проблемы сердечно-сосудистых заболеваний 2022;11(4):118-129. https://doi.org/10.17802/2306-1278-2022-11-4-118-129

292. Obokata M, Reddy YNV, Melenovsky V, et al. Deterioration in right ventricular structure and function over time in patients with heart failure and preserved ejection fraction. Eur Heart J 2019; 40:689–697. https://doi.org/10.1093/eurheartj/ehy809

293. Lewis GD, Shah R, Shahzad K, et al. Sildenafil improves exercise capacity and quality of life in patients with systolic heart failure and secondary pulmonary hypertension. Circulation 2007; 116:1555–1562. https://doi.org/10.1161/circulationaha.107.716373

294. Wu X, Yang T, Zhou Q, Li S, Huang L. Additional use of a phosphodiesterase 5 inhibitor in patients with pulmonary hypertension secondary to chronic systolic heart failure: a meta-analysis. Eur J Heart Fail 2014; 16:444–453. https://doi.org/10.1002/ejhf.47

295. Guazzi M, Samaja M, Arena R, et al. Long-term use of sildenafil in the therapeutic management of heart failure. J Am Coll Cardiol. 2007 Nov 27;50(22):2136-44. https://doi.org/10.1016/j.jacc.2007.07.078

296. Obokata M, Reddy YNV, Shah SJ, et al. Effects of interatrial shunt on pulmonary vascular function in heart failure with preserved ejection fraction. J Am Coll Cardiol 2019;74:2539–2550. https://doi.org/10.1016/j.jacc.2019.08.1062

297. Guazzi M, Vicenzi M, Arena R, Guazzi MD. Pulmonary hypertension in heart failure with preserved ejection fraction: a target of phosphodiesterase-5 inhibition in a 1-year study. Circulation. 2011 Jul 12;124(2):164-174. https://doi.org/10.1161/circulationaha.110.983866

298. Opitz CF, Hoeper MM, Gibbs JS, et al. Pre-capillary, combined, and post-capillary pulmonary hypertension: a pathophysiological continuum. J Am Coll Cardiol 2016; 68:368–378. https://doi.org/10.1016/j.jacc.2016.05.047

299. Hoendermis ES, Liu LC, Hummel YM, et al. Effects of sildenafil on invasive haemodynamics and exercise capacity in heart failure patients with preserved ejection fraction and pulmonary hypertension: a randomized controlled trial. Eur Heart J 2015;36:2565–2573. https://doi.org/10.1093/eurheartj/ehv336

300. Dachs TM, Duca F, Rettl R, et al. Riociguat in pulmonary hypertension and heart failure with preserved ejection fraction: the haemoDYNAMIC trial. Eur Heart J 2022; 43(36):3402-3413. https://doi.org/10.1093/eurheartj/ehac389

301. Selim AM, Wadhwani L, Burdorf A, et al. Left ventricular assist devices in pulmonary hypertension group 2 with significantly elevated pulmonary vascular resistance: a bridge to cure. Heart Lung Circ 2019; 28(6):946-952. https://doi.org/10.1016/j.hlc.2018.04.299

302. Bonderman D, Ghio S, Felix SB, et al. Riociguat for patients with pulmonary hypertension caused by systolic left ventricular dysfunction: a phase IIb double-blind, randomized, placebo-controlled, dose-ranging hemodynamic study. Circulation 2013; 128(5):502-11. https://doi.org/10.1161/circulationaha.113.001458

303. O’Sullivan CJ, Wenaweser P, Ceylan O, et al. Effect of pulmonary hypertension hemodynamic presentation on clinical outcomes in patients with severe symptomatic aortic valve stenosis undergoing transcatheteraortic valve implantation: insights from the new proposed pulmonary hypertension classification. Circ Cardiovasc Interv 2015;8:e002358. https://doi.org/10.1161/circinterventions.114.002358

304. Tigges E, Blankenberg S, von Bardeleben RS, et al. Implication of pulmonary hypertension in patients undergoing MitraClip therapy: results from the German transcatheter mitral valve interventions (TRAMI) registry. Eur J Heart Fail 2018; 20:585–594. https://doi.org/10.1002/ejhf.864

305. Gaemperli O, Moccetti M, Surder D, et al. Acute haemodynamic changes after percutaneous mitral valve repair: relation to mid-term outcomes. Heart 2012; 98:126–132. https://doi.org/10.1136/heartjnl-2011-300705

306. Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS Guidelines for the management of valvular heart disease. Eur Heart J 2022;43:561–632. https://doi.org/10.1093/eurheartj/ehab395

307. Schneider SR, Mayer LC, Lichtblau M, et al. Effect of a day-trip to altitude (2500 m) on exercise performance in pulmonary hypertension: randomized crossover trial. ERJ Open Res 2021;7:00314-02021. https://doi.org/10.1183/23120541.00314-2021

308. Groth A, Saxer S, Bader PR, et al. Acute hemodynamic changes by breathing hypoxic and hyperoxic gas mixtures in pulmonary arterial and chronic thromboembolic pulmonary hypertension. Int J Cardiol 2018; 270:262–267. https://doi.org/10.1016/j.ijcard.2018.05.127

309. Zeder K, Avian A, Bachmaier G, et al. Elevated pulmonary vascular resistance predicts mortality in COPD patients. Eur Respir J 2021; 58: 2100944. https://doi.org/10.1183/13993003.00944-2021

310. Nadrous HF, Pellikka PA, Krowka MJ, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 2005; 128: 2393-2399. https://doi.org/10.1378/chest.128.4.2393

311. Olsson KM, Hoeper MM, Pausch C, et al. Pulmonary vascular resistance predicts mortality in patients with pulmonary hypertension associated with interstitial lung disease: results from the COMPERA registry. Eur Respir J 2021; 58: 2101483. https://doi.org/10.1183/13993003.01483-2021

312. Chaouat A, Bugnet AS, Kadaoui N, et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2005; 172: 189–194. https://doi.org/10.1164/rccm.200401-006oc

313. Lettieri CJ, Nathan SD, Barnett SD, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006; 129: 746–752. https://doi.org/10.1378/chest.129.3.746

314. Thabut G, Dauriat G, Stern JB, et al. Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest 2005; 127: 1531–1536. https://doi.org/10.1378/chest.127.5.1531

315. Dawes TJW, McCabe C, Dimopoulos K, et al. Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study. Respirology. 2023; 28:262-72. https://doi.org/10.1111/resp.14378

316. Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013; 158: 641–649. https://doi.org/10.7326/0003-4819-158-9-201305070-00003

317. Gou Q, Shi R, Zhang X, Meng Q, Li X, Rong X, Gawa Z, Zhuoma N, Chen X. The Prevalence and Risk Factors of High-Altitude Pulmonary Hypertension Among Native Tibetans in Sichuan Province, China. High Alt Med Biol. 2020;21(4):327-335. https://doi.org/10.1089/ham.2020.0022

318. Сарыбаев А.Ш., Сыдыков А.С., Сартмырзаева М.А., др. Высокогорная лёгочная гипертония («болезнь Миррахимова»). Евразийский кардиологический журнал 2016;(4):76-83. https://doi.org/10.38109/2225-1685-2016-4-76-83

319. Sime F, Penaloza D, Ruiz L. Bradycardia, increased cardiac output, and reversal of pulmonary hypertension in altitude natives living at sea level. Br Heart J. 1971;33:647-657. https://doi.org/10.1136/hrt.33.5.647

320. Aldashev AA, Kojonazarov BK, Amatov TA, et al. Phosphodiesterase type 5 and high altitude pulmonary hypertension. Thorax 2005;60:683-687. https://doi.org/10.1136/thx.2005.041954

321. Antezana AM, Antezana G, Aparicio O, et al. Pulmonary hypertension in high-altitude chronic hypoxia: response to nifedipine. Eur Respir J 1998;12:1181-1185. https://doi.org/10.1183/09031936.98.12051181

322. Konstantinides SV, Meyer G, Becattini C, et al. 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS).Eur Heart J 2020;41:543–603. https://doi.org/10.1093/eurheartj/ehz405

323. Swietlik EM, Ruggiero A, Fletcher AJ, et al. Limitations of resting haemodynamics in chronic thromboembolic disease without pulmonary hypertension. Eur Respir J 2019;53:1801787. https://doi.org/10.1183/13993003.01787-2018

324. Wensel R, Francis DP, Meyer FJ, et al. Incremental prognostic value of cardiopulmonary exercise testing and resting haemodynamics in pulmonary arterial hypertension. Int J Cardiol 2013 Aug 20;167(4):1193–1198. https://doi.org/10.1016/j.ijcard.2012.03.135

325. Badagliacca R, Rischard F, Giudice FL, et al. Incremental value of cardiopulmonary exercise testing in intermediate-risk pulmonary arterial hypertension. J Heart Lung Transplant 2022;41:780–790. https://doi.org/10.1016/j.healun.2022.02.021

326. Jenkins DP, Biederman A, D"Armini AM, et al. Operability assessment in CTEPH: Lessons from the CHEST-1 study. J Thorac Cardiovasc Surg. 2016;152(3):669–674. https://doi.org/10.1016/j.jtcvs.2016.02.062

327. Чернявский А.М., Едемский А.Г., Новикова Н.В., др. Хирургическое лечение хронической тромбоэмболической лёгочной гипертензии./ Национальный медицинский исследовательский центр имени Е.Н. Мешалкина; под общей редакцией А.М. Чернявского. — Новосибирск: НМИЦ: Издательство СО РАН, 2019.317 с. ISBN: 978-5-7692-1665-7

328. Araszkiewicz A, Darocha S, Pietrasik A, et al. Balloon pulmonary angioplasty for the treatment of residual or recurrent pulmonary hypertension after pulmonary endarterectomy. Int J Cardiol. 2019;278: 232–237. https://doi.org/10.1016/j.ijcard.2018.10.066

329. Madani MM, Auger WR, Pretorius V, et al. Pulmonary endarterectomy: recent changes in a single institution’s experience of more than 2,700 patients. Ann Thorac Surg 2012;94:97–103. https://doi.org/10.1016/j.athoracsur.2012.04.004

330. Lankeit M, Krieg V, Hobohm L, et al. Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2018;37:250–258. https://doi.org/10.1016/j.healun.2017.06.011

331. Акчурин Р.С., Мершин К.В., Табакьян Е.А., др. Хирургическое лечение хронической тромбоэмболической лёгочной гипертензии: современные тенденции и собственный опыт. Евразийский Кардиологический Журнал. 2016; 2:40-47. https://doi.org/10.38109/2225-1685-2016-2-40-47

332. D’Armini AM, Morsolini M, Mattiucci G, Grazioli V, Pin M, Valentini A, et al. Pulmonary endarterectomy for distal chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 2014;148:1005–1011. https://doi.org/10.1016/j.jtcvs.2014.06.052

333. Newnham M, Bunclark K, Abraham N, et al. CAMPHOR score: patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2020;56:1902096. https://doi.org/10.1183/13993003.02096-2019

334. Чернявский А.М., Едемский А.Г., Чернявский М.А., др. Пятилетние результаты хирургического лечения пациентов с хронической постэмболической лёгочной гипертензией Хирургия. Журнал им. Н.И. Пирогова. 2017;2:21-24. https://doi.org/10.17116/hirurgia2017221-24

335. Taboada D, Pepke-Zaba J, Jenkins DP, et al. Outcome of pulmonary endarterectomy in symptomatic chronic thromboembolic disease. Eur Respir J. 2014;44:1635–1645. https://doi.org/10.1183/09031936.00050114

336. Quadery SR, Swift AJ, Billings CG, et al. The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2018;52:1800589. https://doi.org/10.1183/13993003.00589-2018

337. Jaïs X, Brenot P, Bouvaist H, et al. Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study. Lancet Respir Med. 2022;10(10):961-971. https://doi.org/10.1016/s2213-2600(22)00214-4

338. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated chronic thromboembolic pulmonary hypertension patients. Eur Respir J. 2018;52:1800248. https://doi.org/10.1183/13993003.00248-2018

339. Benza RL, Farber HW, Frost A, et al. REVEAL risk score in patients with chronic thromboembolic pulmonary hypertension receiving riociguat. J Heart Lung Transplant. 2018;37:836–843. https://doi.org/10.1016/j.healun.2018.02.015

340. Humbert M, Farber HW, Ghofrani HA, et al. Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53:1802004. https://doi.org/10.1183/13993003.02004-2018

341. Bunclark K, Newnham M, Chiu YD, et al. A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension. J Thromb Haemost. 2020;18:114–122. https://doi.org/10.1111/jth.14649

342. Humbert MS, Simonneau G, Pittrow D, et al. Oral anticoagulants (NOAC and VKA) in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2022;41:716–721. https://doi.org/10.1016/j.healun.2022.02.002

343. Валиева З.С., Мартынюк Т.В. Выбор специфической и антикоагулянтной терапии у пациентов с впервые выявленной хронической тромбоэмболической легочной гипертензией в зависимости от статуса операбельности. Российский кардиологический журнал. 2023;28(3):5231. https://doi.org/10.15829/1560-4071-2023-5231

344. Ordi-Ros J, Saez-Comet L, Perez-Conesa M, et al. Rivaroxaban versus vitamin K antagonist in antiphospholipid syndrome: a randomized noninferiority trial. Ann Intern Med 2019;171:685–694. https://doi.org/10.7326/m19-0291

345. Pengo V, Denas G, Zoppellaro G, et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood 2018;132:1365–1371. https://doi.org/10.1182/blood-2018-04-848333

346. Ghofrani HA, Simonneau G, D’Armini AM, et al. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med. 2017;5:785–794. https://doi.org/10.1016/s2213-2600(17)30305-3

347. Reesink HJ, Surie S, Kloek JJ, et al. Bosentan as a bridge to pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg. 2010;139:85–91

348. Reichenberger F, Voswinckel R, Enke B, et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hyper- tension. Eur Respir J. 2007;30:922–927. https://doi.org/10.1183/09031936.00039007

349. Данилов Н.М., Матчин Ю.Г., Мартынюк Т.В. и др. Баллонная ангиопластика лёгочных артерий при неоперабельной хронической тромбоэмболической лёгочной гипертензии. Сonsilium Medicum 2016;5:59-61. https://consilium.orscience.ru/2075-1753/article/view/94466

350. Mahmud E, Behnamfar O, Ang L,et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Interv Cardiol Clin 2018;7:103–117. https://doi.org/10.1016/j.iccl.2017.09.003

351. Ogawa A, Matsubara H. After the dawn-balloon pulmonary angioplasty for patients with chronic thromboembolic pulmonary hypertension. Circ J 2018;82:1222-1230. https://doi.org/10.1253/circj.cj-18-0258

352. Ogawa A, Satoh T, Fukuda T, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: results of a multicenter registry. Circ Cardiovasc Qual Outcomes. 2017;10:e004029. https://doi.org/10.1161/circoutcomes.117.004029

353. Wiedenroth CB, Ghofrani HA, Adameit MSD, et al. Sequential treatment with riociguat and balloon pulmonary angioplasty for patients with inoperable chronic thromboembolic pulmonary hypertension. Pulm Circ 2018; 8:2045894018783996. https://doi.org/10.1177/2045894018783996

354. Fukui S, Ogo T, Morita Y, et al. Right ventricular reverse remodelling after balloon pulmonary angioplasty. Eur Respir J 2014;43:1394–1402. https://doi.org/10.1183/09031936.00012914

355. Fukuda K, Hiroshi Date H, Dоi S, et al. Guidelines for the Treatment of Pulmonary Hypertension (JCS 2017/JPCPHS 2017). Circ J 2019;83(4):842-945. https://doi.org/10.1253/circj.cj-66-0158

356. Яровой С.Ю., Данилов Н.М., Матчин Ю.Г., Чазова И.Е. Структурные изменения легочных артерий после транслюминальной баллонной ангиопластики у пациентов с хронической тромбоэмболической легочной гипертензией. Евразийский Кардиологический Журнал. 2020;(4):12-20. https://doi.org/10.38109/2225-1685-2020-4-12-20

357. Andreassen AK, Ragnarsson A, Gude E, et al. Balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension. Heart 2013;99:1415–1420. https://doi.org/10.1136/heartjnl-2012-303549

358. Kurzyna M, Darocha S, Pietura R, Pietrasik A, Norwa J, Mańczak R, et al. Changing the strategy of balloon pulmonary angioplasty resulted in a reduced complication rate in patients with chronic thromboembolic pulmonary hypertension. A single-centre European experience. Kardiol Pol 2017;75:645–654. https://doi.org/10.5603/kp.a2017.0091

359. Olsson KM, Wiedenroth CB, Kamp JC, Breithecker A, Fuge J, Krombach GA, et al. Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension: the initial German experience. Eur Respir J 2017;49:1602409. https://doi.org/10.1183/13993003.02409-2016

360. Godinas L, Bonne L, Budts W, et al. Balloon pulmonary angioplasty for the treatment of nonoperable chronic thromboembolic pulmonary hypertension: single-center experience with low initial complication rate. J Vasc Interv Radiol 2019;30:1265-1272. https://doi.org/10.1016/j.jvir.2019.03.023

361. Lang IM, Andreassen AK, Andersen A, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function. Eur Heart J 2023;44(29):2659-2671. https://doi.org/10.1093/eurheartj/ehad413

362. Shinkura Y, Nakayama K, Yanaka K, et al. Extensive revascularisation by balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension beyond haemodynamic normalisation. EuroIntervention 2018;13:2060-2068. https://doi.org/10.4244/eij-d-17-00157

363. Douketis JD, Spyropoulos AC, Kaatz S, et al. Perioperative bridging anticoagulation in patients with atrial fibrillation. N Engl J Med 2015;373:823-833. https://doi.org/10.1056/nejmoa1501035

364. Danilov NM, Matchin YuG, Sagaydak OV, et al. Antecubital vein access for balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension: safe new approach. Russian Open Medical Journal 2019;8:e0107. https://doi.org/10.15275/rusomj.2019.0107

365. Данилов Н.М., Яровой С.Ю., Елфимова Е.М., др. Клинические и рентгенологические аспекты реперфузионного отёка лёгких после транслюминальной баллонной ангиопластики лёгочных артерий у пациентов с хронической тромбоэмболической лёгочной гипертензией. Системные гипертензии. 2022;19(3):23-30. https://doi.org/10.38109/2075-082X-2022-3-23-30

366. Jensen KW, Kerr KM, Fedullo PF, et al. Pulmonary hypertensive medical therapy in chronic thromboembolic pulmonary hypertension before pulmonary thromboendarterectomy. Circulation 2009;120(13):1248-54. https://doi.org/10.1161/circulationaha.109.865881

367. Валиева З.С., Мартынюк Т.В., Наконечников С.Н., Чазова И.Е. Характеристика пациентов с хронической тромбоэмболической лёгочной гипертензией по данным российского национального регистра. Терапевтический архив. 2021;93(9):1058-1065. https://doi.org/10.26442/00403660.2021.09.201037

368. Ni JR, Yan PJ, Liu SD, et al. Diagnostic accuracy of transthoracic echocardiography for pulmonary hypertension: a systematic review and meta-analysis. BMJ Open. 2019; 9(12):e033084. https://doi.org/10.1136/bmjopen-2019-033084

369. Gall H, Yogeswaran A, Fuge J, et al. Validity of echocardiographic tricuspid regurgitation gradient to screen for new definition of pulmonary hypertension. EClinicalMedicine. 2021;34:100822. https://doi.org/10.1016/j.eclinm.2021.100822

370. D'Alto M., Di Maio M., Romeo E, et al. Echocardiographic probability of pulmonary hypertension: a validation study. Eur Respir J 2022;60:2102548. https://doi.org/10.1183/13993003.02548-2021

371. Sydykov A, Mamazhakypov A, Maripov A, et al. Pulmonary Hypertension in Acute and Chronic High Altitude Maladaptation Disorders. Int J Environ Res Public Health. 2021;18(4):1692. https://doi.org/10.3390/ijerph18041692

372. Soria R, Egger M, Scherrer U, et al. Pulmonary artery pressure and arterial oxygen saturation in people living at high or low altitude: systematic review and meta-analysis. J Appl Physiol 2016;121(5):1151-1159. https://doi.org/10.1152/japplphysiol.00394.2016

373. Maripov A, Mamazhakypov A, Karagulova G, et al. High altitude pulmonary hypertension with severe right ventricular dysfunction. Int J Cardiol. 2013;168(3):e89-e90. https://doi.org/10.1016/j.ijcard.2013.07.129

374. Grover RF, Vogel JH, Voigt GC, et al. Reversal of high altitude pulmonary hypertension. Am J Cardiol 1966;18:928-932. https://doi.org/10.1016/0002-9149(66)90443-7

375. Maripov A, Muratali Uulu K, Satybaldyev S, Kushubakova N, Sarybaev A. Reversal of pulmonary hypertension in a patient with chronic mountain sickness after relocation to low altitude. J Clin Diagn Res. 2021 y;15(5):OD09-OD10. http://dx.doi.org/10.7860/JCDR/2021/48309.14886

376. Singh I, Khanna PK, Lal M, et al. High-altitude pulmonary hypertension. Lancet. 1965;2(7404):146-50. https://doi.org/10.1016/s0140-6736(65)90229-1

377. Richalet JP, Rivera-Ch M, Maignan M, et al. Acetazolamide for Monge's disease: efficiency and tolerance of 6-month treatment. Am J Respir Crit Care Med. 2008;177(12):1370-6. https://doi.org/10.1164/rccm.200802-196oc

378. Ishikura K, Yamada N, Ito M, et al. Beneficial acute effects of rho-kinase inhibitor in patients with pulmonary arterial hypertension. Circ. J. Off. J. Jpn. Circ. Soc. 2006;70:174-178. https://doi.org/10.1253/circj.70.174

379. Kojonazarov B, Myrzaakhmatova A, Sooronbaev T, et al. Effects of fasudil in patients with high-altitude pulmonary hypertension. Eur Respir J. 2012;39:496-498. https://doi.org/10.1183/09031936.00095211

380. Sommer N, Ghofrani H-A, Pak O, et al. Current and future treatments of pulmonary arterial hypertension. Br J Pharmacol. 2021;178:6-30. https://doi.org/10.1111/bph.15016

381. Grimminger F, Schermuly RT, Ghofrani H-A. Targeting non-malignant disorders with tyrosine kinase inhibitors. Nat Rev Drug Discov. 2010;9:956-970. https://doi.org/10.1038/nrd3297