Статья
Легочная гипертензия, в том числе хроническая тромбоэмболическая легочная гипертензия. Клинические рекомендации 2024
Российское кардиологическое общество (РКО)При участии: Ассоциации сердечно-сосудистых хирургов России (АССХ), Евразийской ассоциации кардиологов (ЕАК), Российского медицинского общества по артериальной гипертонии (РМОАГ), Российского респираторного общества (РРО), Ассоциации анестезиологов и реаниматологов, Российского общества рентгенологов и радиологов (РОРР), Ассоциации ревматологов России.Одобрено Научно-практическим Советом Минздрава Российской Федерации (12.09.2024).
1. Sackett DL, Rosenberg WM, Gray JA, et al. Evidence based medicine: what it is and what it isn’t? BMJ. 1996;312(7023):71-72.
2. Galiè 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): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67-119.
3. Андреева Н. С., Реброва О. Ю., Зорин Н. А. др. Системы оценки достоверности научных доказательств и убедительности рекомендаций: сравнительная характеристика и перспективы унификации. Медицинские технологии. Оценка и выбор. 2012;4:10-24.
4. 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.
5. Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1801889. doi:10.1183/13993003.01889-2018.
6. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53:1801913.
7. 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):3618-731.
8. 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):888-94.
9. Maron BA, Brittain EL, Hess E, et al. Pulmonary vascular resistance and clinical out-comes in patients with pulmonary hypertension: a retrospective cohort study. Lancet Respir Med. 2020;8:873-84.
10. Авдеев С. Н., Барбараш О. Л., Баутин А. Е. и др. Легочная гипертензия, в том числе хроническая тромбоэмболическая легочная гипертензия. Клинические рекомендации 2020. Российский кардиологический журнал. 2021;26(12):4683. doi:10.15829/1560-4071-2021-4683.
11. Humbert M, Guignabert C, Bonnet S, et al. Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives. Eur Respir J. 2019;53(1):1801887.
12. 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.
13. Frost A, Badesch D, Simon JR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019;53:1801904.
14. Чазова И. Е., Мартынюк Т. В., Валиева З. С. др. Евразийские клинические рекомендации по диагностике и лечению легочной гипертензии (2019). Евразийский Кардиологический Журнал. 2020;1:78-122.
15. 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.
16. Hurdman J, Condliffe R, Elliot CA, et al. Pulmonary hypertension in COPD: results from the ASPIRE registry. Eur Respir J. 2013;41:1292-301.
17. Nathan SD, Barbera JA, Gaine SP, et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J. 2019;53:1801914.
18. Simonneau G, Torbicki A, Dorfmüller P, Kim N. The pathophysiology of chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017;26(143):160112.
19. Hoeper MM, Humbert M, Souza R, et al. A global view of pulmonary hypertension. Lancet Respir Med. 2016;4:306-22.
20. 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.
21. 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. doi:10.1155/2020/6836973.
22. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107(2):216-23.
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.
24. 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):156-63.
25. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137(2):376-87.
26. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2):871-80.
27. Certain MC, Chaumais MC, Jais X, et al. Characteristics and long-term outcomes of pulmonary venoocclusive disease induced by mitomycin C. Chest. 2021;159:1197-207.
28. Cornet L, Khouri C, Roustit M, et al. Pulmonary arterial hypertension associated with protein kinase inhibitors: a pharmacovigilance-pharmacodynamic study. Eur Respir J. 2019;53:1802472.
29. Weatherald J, Bondeelle L, Chaumais MC, et al. Pulmonary complications of BcrAbl tyrosine kinase inhibitors. Eur Respir J. 2020;56:2000279.
30. Chen SC, Dastamani A, Pintus D, et al. Diazoxide-induced pulmonary hypertension in hyperinsulinaemic hypoglycaemia: Recommendations from a multicentre study in the UK. Clin Endocrinol (Oxf). 2019;91:770-5.
31. Global Burden of Disease Study Collaborators. Global, regional, and national incidence, prevalence, and years lived with disability for 301 acute and chronic diseases and injuries in 188 countries, 1990-2013: a systematic analysis for the Global Burden of Disease Study 2013. Lancet. 2015;386:743-800.
32. Mehra P, Mehta V, Sukhija R, et al. Pulmonary hypertension in left heart disease. Arch Med Sci. 2019;15(1):262-73.
33. Tichelbacker T, Dumitrescu D, Gerhardt F, et al. Pulmonary hypertension and valvular heart disease. Herz. 2019;44:491-501.
34. Naeije R. Pulmonary hypertension at high altitude. Eur Respir J. 2019;53(6):1900985.
35. Delcroix M, Torbicki A, Gopalan D, et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J. 2021;57(6):2002828.
36. Kramm T, Wilkens H, Fuge J, et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol. 2018;107(7):548-53. doi:10.1007/s00392-018-1215-5.
37. Boucly A, Cottin V, Nunes H, et al. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension. Eur Respir J. 2017;50:1700465.
38. 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;40:3297-317.
39. Чазова И. Е., Мартынюк Т. В., Валиева З. С. и др. Евразийские рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (2020). Евразийский Кардиологический Журнал. 2021;1:6-43.
40. Бокерия Л. А., Горбачевский С. В., Шмальц А. А. Легочная артериальная гипертензия, ассоциированная с врожденными пороками сердца у взрослых (клиническая рекомендация). Грудная и сердечно-сосудистая хирургия. 2017;59(2):135-47.
41. 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 легочной гипертензией по данным Российского регистра. Терапевтический архив. 2019;91(9):77-87.
42. Чазова И. Е., Авдеев С. Н., Царева Н. А. др. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевтический архив. 2014;9:4-23.
43. Чазова И. Е., Горбачевский С. В., Мартынюк Т. В. и др. Евразийские рекомендации по диагностике и лечению легочной гипертензии, ассоциированной с врожденными пороками сердца у взрослых (2021). Евразийский кардиологический журнал. 2022;2:6-70.
44. Мартынюк Т. В. Легочная гипертензия: диагностика и лечение. Москва: ООО "Медицинское информационное агентство", 2018. 304 с. ISBN: 978-5-6040008-0-9.
45. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369(9):809-18.
46. Чазова И. Е., Архипова О. А., Мартынюк Т. В. Легочная артериальная гипертензия в России: анализ шестилетнего наблюдения по данным Национального регистра. Терапевтический архив. 2019;91(1):10-31.
47. Хроническая тромбоэмболическая легочная гипертензия. Руководство для врачей. Под ред. Т. В. Мартынюк, акад. РАН И. Е. Чазовой. Москва: ООО "Медицинское информационное агентство", 2023. 416 с.
48. Helmersen D, Provencher S, Hirsch AM, et al. Diagnosis of chronic thromboembolic pulmonary hypertension: A Canadian Thoracic Society clinical practice guideline update. Can J Respir Crit Care Sleep Med. 2019;3:177-98.
49. Алокова Ф. Х., Блинова Е. В., Сахнова Т. А. и др. Изменения электрокардиограммы в 12 отведениях у больных с идиопатической легочной гипертензией. Медицинский алфавит. 2018;36(4):29-35.
50. Bonderman D, Wexberg P, Martischnig AM, et al. A noninvasive algorithm to exclude pre-capillary pulmonary hypertension. Eur Respir J. 2011;37:1096-103.
51. Rich JD, Thenappan T, Freed B, et al. QTc prolongation is associated with impaired right ventricular function and predicts mortality in pulmonary hypertension. Int J Cardiol. 2013;167:669-76.
52. Mercurio V, Peloquin G, Bourji KI, et al. Pulmonary arterial hypertension and atrial arrhythmias: incidence, risk factors, and clinical impact. Pulm Circ. 2018;8(2): 2045894018769874.
53. 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;128:21-6.
54. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005;26:948-68.
55. Wright LM, Dwyer N, Celermajer D, et al. Follow-Up of Pulmonary Hypertension With Echocardiography. JACC Cardiovasc Imaging. 2016;9(6):733-46.
56. 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.
57. Lang RM, Badano LP, Mor-Avi V, et al. Recommendations for cardiac chamber quanпults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. EurHeart J Cardiovasc Imaging. 2015;16:233-70.
58. Белевская А. А., Дадачева З. Х., Саидова М. А. и др. Возможности эхокардиографии в диагностике легочной гипертензии и оценке ремоделирования сердца. Лечебное дело. 2015;1:111-21.
59. Неклюдова Г. В., Науменко Ж. К. Эхокардиография при диагностике легочной гипертензии. Практическая пульмонология. 2015;2:48-56.
60. Казымлы А. В., Рыжков А. В., Симакова М. А. и др. Значение двухмерной эхокардиографии в оценке степени тяжести пациентов с легочной гипертензией. Кардиология. 2016;56(1):25-30.
61. Balakrishnan B, Owens B, Hayes R, Wen S. A Systematic Review of Echocardiographic Parameters for the Screening of Pulmonary Hypertension: What Are the Odds? Cureus. 2022;14(12):e32185. doi:10.7759/cureus.32185.
62. D’Alto M, Di Maio M, Romeo E, et al. Echocardiographic probability of pulmonary hypertension: a validation study. Eur Respir J. 2022;60:2102548.
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):1667-73.
64. 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):988-93.
65. 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):10-8.
66. 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.
67. 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.
68. 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.
69. Gall H, Yogeswaran A, Fuge J, et al. Validity of echocardiographic tricuspid regurgitation gradient to screen for new definition of pulmonary hypertension. E Clinical Medicine. 2021;34:100822.
70. 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.
71. 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):2778-88.
72. 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.
73. 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;46:2429-51.
74. 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;48:680-4.
75. Архипова О. А., Мартынюк Т. В., Самойленко Л. Е. и др. Перфузионная сцинтиграфия легких у больных с легочной гипертензией различной этиологии. Евразийский кардиологический журнал. 2015;4:21-5.
76. 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;46:1693-7.
77. 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;28:5100-110.
78. Czerner CP, Schoenfeld C, Cebotari S, et al. Perioperative CTEPH patient monitoring with 2D phase-contrast MRI reflects clinical, cardiac and pulmonary perfusion changes after pulmonary endarterectomy. PLOS ONE. 2020;15(9):e023817б.
79. 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;30:4857-64.
80. 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.
81. Swift AJ, Dwivedi K, Johns C, et al. Diagnostic accuracy of CT pulmonary angiography in suspected pulmonary hypertension. Eur Radiol. 2020;30:4918-29.
82. 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.
83. 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;22:310-7.
84. 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;38:731-8.
85. 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.
86. 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;243:538-43.
87. Веселова Т. Н., Демченкова А. Ю., Ен М. Ю. и др. Оценка перфузии легких у больных хронической тромбоэмболической легочной гипертензией методами компьютерной томографии и сцинтиграфии. REJR. 2022;12(2):94-103. doi:10.21569/2222-7415-2022-12-2-94-103.
88. 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;5:125-34.
89. 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):178-93.
90. 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.
91. Amzulescu MS, De Craene M, Langet H, et al. Myocardial strain imaging: review of general principles, validation, and sources of discrepancies. Eur Heart J Cardiovasc Imaging. 2019; 20(6):605-19.
92. Ray JC, Burger C, Mergo P, et al. Pulmonary arterial stiffness assessed by cardiovascular magnetic resonance imaging is a predictor of mild pulmonary arterial hypertension. Int J Cardiovasc Imaging. 2019;35(10):1881-92.
93. Ohno Y, Yoshikawa T, Kishida Y, et al. Unenhanced and contrast-enhanced MR angiography and perfusion imaging for suspected pulmonary thromboembolism. Am J Roentgenol. 2017;208(3):517-30.
94. 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.
95. Swift AJ, Rajaram S, Hurdman J. et al. Noninvasive estimation of PA pressure, flow, and resistance with CMR imaging: derivation and prospective validation study from the ASPIRE registry. JACC Cardiovasc Imaging. 2013;6:1036-47.
96. Naeije R. Hepatopulmonary syndrome and portopulmonary hypertension. SwissMed Wkly. 2003;133:163-9.
97. Rosenkranz S, Howard LS, Gomberg-Maitland M, Hoeper MM. Systemic consequences of pulmonary hypertension and right-sided heart failure. Circulation. 2020;141:678-93.
98. Шмальц А. А. Динамика дистанции шестиминутной ходьбы как показатель эффективности специфических легочных вазодилататоров. Рациональная Фармакотерапия в Кардиологии. 2022;18(3):342-9.
99. 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):1192-201.
100. Таран И. Н., Валиева З. С., Мартынюк Т. В. и др. Вклад спировелоэргометрии в диагностический алгоритм обследования больных с легочной артериальной гипертензией. Медицинский алфавит. Больница (кардиология). 2016;3:19-23.
101. Казымлы А. В., Березина А. В., Рыжков А. В. и др. Кардиопульмональное тестирование как метод оценки степени тяжести пациентов с прекапиллярной легочной гипертензией. Кардиология. 2014;54(12):22-8.
102. Dumitrescu D, Nagel C, Kovacs G, et al. Cardiopulmonary exercise testing for detecting pulmonary arterial hypertension in systemic sclerosis. Heart. 2017;103:774-82.
103. Boerrigter BG, Bogaard HJ, Trip P, et al. Ventilatory and cardiocirculatory exercise profiles in COPD: the role of pulmonary hypertension. Chest. 2012;142:1166-74.
104. 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;48:2546-52.
105. D’Alto M, Dimopoulos K, Coghlan JG, et al. Right heart catheterization for the diagnosis of pulmonary hypertension: controversies and practical issues. Heart Fail Clin. 2018; 14:467-77.
106. Kovacs G, Avian A, Olschewski A, Olschewski H. Zero reference level for right heart catheterisation. Eur Respir J. 2013;42:1586-94.
107. 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):23-8.
108. Шмальц А. А., Мартынюк Т. В., Наконечников С. Н. Расчет гемодинамики методом Фика при легочной гипертензии, ассоциированной с врожденными пороками сердца. Евразийский кардиологический журнал. 2022;(2):96-102.
109. 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;2:1090-9.
110. 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.
111. Vachiery JL, Tedford RJ, Rosenkranz S, et al. Pulmonary hypertension due to left heart disease. Eur Respir J. 2019;53:1801897.
112. Kubiak GM, Ciarka A, Biniecka M, et al. Right heart catheterization — background, physiological basics, and clinical implications. J Clin Med. 2019;8:1331.
113. 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.
114. 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.
115. Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005;111:3105-11.
116. Hoeper MM, Olschewski H, Ghofrani HA, et al. A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension. German PPH study group. J Am Coll Cardiol. 2000;35:176-82.
117. Opitz C. Assessment of the vasodilator response in primary pulmonary hypertension Comparing prostacyclin and iloprost administered by either infusion or inhalation. Eur Heart J. 2003;24(4):356-65.
118. 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.
119. Wilkens Н, Konstantinides S, Lang IM, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): Updated recommendations from the Cologne Consensus Conference 2018. Int. J. Cardiol. 2018;272:69-78.
120. Юрпольская Л. А., Шмальц А. А. Рентгенография, рентгеновская компьютерная и магнитно-резонансная томография при легочной гипертензии, ассоциированной с врожденными пороками сердца. Детские болезни сердца и сосудов. 2021;18(2):83-93.
121. Авдеев С. Н. Легочная гипертензия. Москва: ГЭОТАР-Медиа, 2019. 608 с. ISBN: 978-5-9704-5000-0.
122. Jilwan FN, Escourrou P, Garcia G, et al. High occurrence of hypoxemic sleep respiratory disorders in precapillary pulmonary hypertension and mechanisms. Chest. 2013;143:47-55.
123. Mancuso L, Scordato F, Pieri M, et al. Management of portopulmonary hypertension: new perspectives. World J Gastroenterol. 2013;19: 8252-7.
124. 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;177:108-13.
125. Волков А. В., Мартынюк Т. В. Легочная артериальная гипертензия при системных заболеваниях соединительной ткани: современное состояние проблемы. Научно-практическая ревматология. 2018;56(4):474-86.
126. Weatherald J, Montani D, Jevnikar M, et al. Screening for pulmonary arterial hypertension in systemic sclerosis. Eur Respir Rev. 2019;28:190023.
127. 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.
128. 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;73:1340-9.
129. 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;52:3792-800.
130. 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;63:3522-30.
131. Morrell NW, Aldred MA, Chung WK, et al. Genetics and genomics of pulmonary arterial hypertension. Eur Respir J. 2019;53:1801899.
132. Kiely DG, Lawrie A, Humbert M. Screening strategies for pulmonary arterial hypertension. Eur Heart J Suppl. 2019;21:K9-K20.
133. 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;76:1002-9.
134. 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;39:4175-81.
135. 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.
136. Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50:1700889.
137. 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;159:337-46.
138. 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.
139. 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;27:114-20.
140. Khirfan G, Naal T, Abuhalimeh B, et al. Hypoxemia in patients with idiopathic or heritable pulmonary arterial hypertension. PLoS One. 2018;13:e0191869.
141. Sitbon O, Chin KM, Channick RN, et al. Risk assessment in pulmonary arterial hypertension: insights from the GRIPHON study. J Heart Lung Transpl. 2020;39:300-9.
142. 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. doi:10.1002/14651858.cd011285.pub2.
143. 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):361-9.
144. 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):3826-924.
145. 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;41:1302-7.
146. Thangamathesvaran L, Armenia SJ, Merchant AM. The effect of pulmonary hypertension on inpatient outcomes of laparoscopic procedures. Updates Surg. 2018;70(4):521-8. doi:10.1007/s13304-018-0556-y.
147. Osterberg L, Blaschke T. Adherence to medication. N Engl J Med. 2005;353:487-97.
148. Чазова И. Е., Мартынюк Т. В., Валиева З. С. и др. Оценка бремени хронической тромбоэмболической легочной гипертензии в Российской Федерации. Терапевтический архив. 2018;9:101-9.
149. 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.
150. 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;270:262-7.
151. Grünig, E, Benjamin, N, Krüger, U, et al. General measures and supportive therapy for pulmonary arterial hypertension: Updated recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018;272S:30-6. doi:10.1016/j.ijcard.2018.08.085.
152. 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;4:122-32.
153. Гончарова Н. С., Симакова М. А., Моисеева О. М. Антитромботическая терапия у пациентов с легочной артериальной гипертензией. Артериальная гипертензия. 2019;25(1):25-33.
154. 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;196:251-6.
155. Rhodes CJ, Wharton J, Howard L, et al. Iron deficiency in pulmonary arterial hypertension: a potential therapeutic target. Eur Respir J. 2011;38(6):1453-60.
156. 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.
157. Olsson KM, Fuge J, Brod T, et al. Oral iron supplementation with ferric maltol in patients with pulmonary hypertension. Eur Respir J. 2020;56:2000616.
158. Howard LSGE, He J, Watson GMJ, et al. Supplementation with iron in pulmonary arterial hypertension. Two randomized crossover trials. Ann Am Thorac Soc. 2021;18:981-8.
159. Olsson KM, Nickel NP, Tongers J, Hoeper MM. Atrial flutter and fibrillation in patients with pulmonary hypertension. Int J Cardiol. 2013;167:2300-5.
160. Clozel M, Maresta A, Humbert M. Endothelin receptor antagonists. Handb Exp Pharmacol. 2013;218:199-227.
161. 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;117:3010-9.
162. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346:896-903.
163. Humbert M, Segal ES, Kiely DG, et al. Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J. 2007;30:338-44.
164. Шмальц А. А., Горбачевский С. В. Риоцигуат и силденафил в лечении легочной гипертензии: сходства и различия. Пульмонология. 2016;26(1):85-91.
165. Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353:2148-57.
166. 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;51:1071-8.
167. Galiè N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009;119:2894-903.
168. Чазова И. Е., Мартынюк Т. В., Шмальц А. А. и др. Евразийские рекомендации по диагностике и лечению лёгочной гипертензии (2023). Евразийский Кардиологический Журнал. 2024;1:6-85.
169. 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. 2015;193(1):105-12.
170. Schermuly RT, Janssen W, Weissmann N, et al. Riociguat for the treatment of pulmonary hypertension. Expert Opin Investig Drugs. 2011;20:567-76.
171. 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.
172. 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 openlabel, randomized, long-term extension trial. Lancet Respir Med. 2016;4:361-71.
173. Galiè N, Manes A, Branzi A. Prostanoids for pulmonary arterial hypertension. Am J Respir Med. 2003;2:123-37.
174. Olschewski H. Inhaled iloprost for the treatment of pulmonary hypertension. Eur Respir Rev. 2009;18(111):29-34.
175. Opitz CF, Wensel R, Winkler J, et al. Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J. 2005;26:1895-902.
176. Мартынюк Т. В. Оценка приверженности пациентов с легочной гипертензией лечению ингаляционным илопростом (Вентавис): итоги проспективного многоцентрового неинтервенционного исследования IVENT. Системные гипертензии. 2019;16(2):12-27.
177. Sitbon O, Channick R, Chin KM, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373:2522-33.
178. 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):842-54.
179. Lang IM, Palazzini M. The burden of comorbidities in pulmonary arterial hypertension. Eur Heart J Suppl. 2019;21(Suppl K):K21-K28.
180. 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):1286-95.
181. 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.
182. Kim NH, Fisher M, Poch D, et al. Long-term outcomes in pulmonary arterial hypertension by functional class: a meta-analysis of randomized controlled trials and observational registries. Pulm Circulation. 2020;10(4):2045894020935291.
183. Hung CC, Cheng CC, Huang WC, et al. Management of Pulmonary Arterial Hypertension Patients with World Health Organization Functional Class II. Acta Cardiol Sin. 2020;36(6):583-7.
184. Шмальц А. А., Горбачевский С. В. Возможности и ограничения для начальной комбинированной специфической терапии легочной артериальной гипертензии в Российской Федерации. Терапевтический архив. 2020;92(12):80-5.
185. 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.
186. Sitbon O, Cottin V, Canuet M, et al. Initial combination therapy of macitentan and tadalafil in pulmonary arterial hypertension. Eur Respir J. 2020;56(3):2000673.
187. Grünig E, Jansa P, Fan F, et al. Randomized Trial of Macitentan/Tadalafil Single-Tablet Combination Therapy for Pulmonary Arterial Hypertension. J Am Coll Cardiol. 2024:83(4):473-84.
188. Чазова И. Е., Мартынюк Т. В. от имени авторов. Резолюция экспертного совета по проблеме легочной артериальной гипертензии "Меняя парадигму лечения пациентов с ЛАГ". Терапевтический архив. 2019;91(3):114-6.
189. 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):277-86.
190. Hoeper MM, Leuchte H, Halank M, et al. Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2006;28:691-4.
191. McLaughlin VV, Oudiz RJ, Frost A, et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006;174:1257-63.
192. Badesch DB, Feldman J, Keogh A, et al. ARIES-3: ambrisentan therapy in a diverse population of patients with pulmonary hypertension. Cardiovasc Ther. 2012;30:93-9.
193. 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.
194. 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):105-9.
195. McLaughlin V, Channick RN, Ghofrani HA, et al. Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J. 2015;46(2):405-13.
196. 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. Respiratory medicine. 2021;9(6)573-84.
197. 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.
198. Taran IN, Belevskaya AA, Saidova MA, et al. Initial Riociguat Monotherapy and Transition from Sildenafil to Riociguat in Patients with Idiopathic Pulmonary Arterial Hypertension: Influence on Right Heart Remodeling and Right Ventricular-Pulmonary Arterial Coupling. Lung. 2018;196(6):745-53.
199. Мартынюк Т. В., Шмальц А. А., Горбачевский С. В., Чазова И. Е. Оптимизация специфической терапии легочной гипертензии: возможности риоцигуата. Терапевтический архив. 2021;93(9):1117-24.
200. 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 metaanalysis.Front Cardiovasc Med. 2022;9:977110.
201. Safdar Z, Thakur A, Frost A. Tolerability of switch to macitentan from bosentan in pulmonary arterial hypertension. South Med J. 2017;110:223-8.
202. 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):304-14.
203. 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.
204. Горбачевский С. В., Пурсанов М. Г., Шмальц А. А. и др. Результаты атриосептостомии со стентированием у больных с идиопатической и схожими формами легочной артериальной гипертензии. Грудная и сердечно-сосудистая хирургия. 2019;61(2):100-13.
205. Шмальц А. А., Нишонов Н. А. Атриосептостомия у больных с легочной гипертензией. Грудная и сердечно-сосудистая хирургия. 2015;57(5):18-25.
206. 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.
207. Bartolome SD, Torres F. Severe pulmonary arterial hypertension: stratification of medical therapies, mechanical support, and lung transplantation. Heart Fail Rev. 2016;21(3):347-56.
208. 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.
209. Jentzer JC, Mathier, MA. Pulmonary Hypertension in the Intensive Care Unit. J Intensive Care Med. 2016;31(6):369-85.
210. Горбачевский С. В., Шмальц А. А., Плотникова Л. Р. Легочная гипертензия у детей с врожденными пороками сердца. М.: ООО "Кедр"; 2018, 92 с. ISBN: 978-5-91487-115-1.
211. Барышникова И. Ю., Шмальц А. А. Диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 2. Эхокардиография. Российский вестник перинатологии и педиатрии. 2021;66(6):23-32.
212. 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.
213. Соболев А. В., Шмальц А. А. Эндоваскулярная диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 1. Катетеризация сердца и ангиокардиография. Эндоваскулярная хирургия. 2021;8(3):263-71.
214. Шмальц А. А., Нишонов Н. А. Эндоваскулярная диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 2. Расчет гемодинамики методом Фика. Эндоваскулярная хирургия. 2021;8(4):339-46.
215. 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.
216. Arvind B, Relan J, Kothari SS. "Treat and repair" strategy for shunt lesions: a critical review. Pulm Circ. 2020;10:2045894020917885.
217. Шмальц А. А., Горбачевский С. В. Доказательная база специфических легочных вазодилататоров у взрослых с врожденными пороками сердца. Терапевтический архив. 2021;93(9):1106-16.
218. Гусева Н. Г. Системная склеродермия — мультидисциплинарная проблема. Научно-практическая ревматология. 2011;49(2):10-4.
219. Волков А. В., Мартынюк Т. В., Юдкина Н. Н. и др. Выживаемость пациентов с легочной артериальной гипертонией, ассоциированной с системной склеродермией. Терапевтический архив. 2012;84(5):24-8.
220. 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.
221. McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev. 2012;21(123):8-18.
222. 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.
223. 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.
224. Николаева Е. В., Корсакова Ю. О., Курмуков И. А. и др. Возможности эхокардиографического определения давления в легочной артерии у пациентов с системными заболеваниями соединительной ткани: данные ревматологического экспертного центра. Научно-практическая ревматология. 2015;53(1):51-7.
225. Юдкина Н. Н., Николаева Е. В., Мартынюк Т. В. и др. Клинический субтип системной склеродермии, ассоциированный с легочной артериальной гипертензией. Кардиологический вестник. 2017;12(4):66-75.
226. 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):917-29.
227. 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.
228. Santos-Gomes J, Gandra I, Adão R, et al. An Overview of Circulating Pulmonary Arterial Hypertension Biomarkers. Front Cardiovasc Med. 2022;9:924873.
229. Wang J, Wang Y, Li X, Huang Y, Serum uric acid is associated with disease severity and may predict clinical outcome in patients of pulmonary arterial hypertension secondary to connective tissue disease in Chinese: a single-center retrospective study. BMC Pulm Med. 2020;20(1):272.
230. 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.
231. Конева О. А., Овсянникова О. Б., Старовойтова М. Н. и др. Изолированное снижение диффузионной способности легких при системной склеродермии без легочной артериальной гипертензии: длительное проспективное наблюдение. Пульмонология. 2016;26(6):708-14.
232. Xanthouli P. Improved Survival for Patients with Systemic Sclerosis-associated Pulmonary Arterial Hypertension: For Real? Am J Respir Crit Care Med. 2023;207(3):238-40.
233. Bourji KI, Kelemen BW, Mathai SC, et al. Poor survival in patients with scleroderma and pulmonary hypertension due to heart failure with preserved ejection fraction. Pulm Circ. 2017;7(2):409-20.
234. Chauvelot L, Gamondes D, Berthiller J, et al. Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease. Arthritis Rheumatol. 2021;73(2):295-304.
235. 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(1):57-65.
236. 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):1808-18.
237. Krowka MJ, Miller DP, Barst RJ, et al. Portopulmonary hypertension: a report from the US-based REVEAL Registry. Chest. 2012;141:906-15.
238. Baiges A, Turon F, Simon-Talero M, et al. Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology. 2020;71:658-69.
239. Savale L, Guimas M, Ebstein N, et al. Portopulmonary hypertension in the current era of pulmonary hypertension management. J Hepatol. 2020;73:130-9.
240. Sitbon O, Bosch J, Cottrel 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.
241. Hoeper MM, Meyer K, Rademacher J, et al. Diffusion capacity and mortality in patients with pulmonary hypertension due to heart failure with preserved ejection fraction. JACC Heart Fail. 2016;4:441-9.
242. 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.
243. Lazaro SM, Quezada Loaiza CA, Rodriguez Padial L, et al. Portopulmonary hypertension: prognosis and management in the current treatment era — results from the REHAP registry. Intern Med J. 2021;51:355-65.
244. Opravil M, Sereni D. Natural history of HIV-associated pulmonary arterial hypertension: trends in the HAART era. AIDS (London, England). 2008;22:S35-S40.
245. 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;21:617-24.
246. 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;170:1212-7.
247. 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.
248. Crawford TC, Leary PJ, Fraser CD, et al. Impact of the new pulmonary hypertension definition on heart transplant outcomes: expanding the hemodynamic risk profile. Chest. 2020;157:151-61.
249. Muraru D, Parati G, Badano L. The importance and the challenges of predicting the progression of functional tricuspid regurgitation. JACC Cardiovasc Imaging. 2020;13:1652-4.
250. Andersen MJ, Hwang SJ, Kane GC, et al. Enhanced pulmonary vasodilator reserve and abnormal right ventricular: pulmonary artery coupling in heart failure with preserved ejection fraction. Circ Heart Fail. 2015;8:542-50.
251. Cao JY, Wales KM, Cordina R, et al. Pulmonary vasodilator therapies are of no benefit in pulmonary hypertension due to left heart disease: A meta-analysis. Int J Cardiol. 2018;273:213-20.
252. Алеевская А. М., Выборов О. Н., Грамович В. В., Мартынюк Т. В. Проблемные аспекты легочной гипертензии вследствие патологии левых отделов сердца: фокус на комбинированную пост/прекапиллярную форму. Терапевтический архив. 2020;92(9):54-62.
253. D’Alto M, Romeo E, Argiento P, et al. Clinical relevance of fluid challenge in patients evaluated for pulmonary hypertension. Chest. 2017;151:119-26.
254. Zeder K, Avian A, Bachmaier G, et al. Elevated pulmonary vascular resistance predicts mortality in COPD patients. Eur Respir J. 2021;58: 2100944.
255. Nadrous HF, Pellikka PA, Krowka MJ, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest. 2005;128:2393-9.
256. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746-52.
257. Zisman DA, Lynch JP, Strieter RM, et al. Pulmonary arterial hypertension (PAH) is common in patients with idiopathic pulmonary fibrosis referred for lung transplantation. Am J Respir Crit Care Med. 2005;2:A123.
258. 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.
259. Chaouat A, Bugnet AS, Kadaoui N, et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2005;172:189-94.
260. Vizza CD, Hoeper MM, Huscher D, et al. Pulmonary hypertension in patients with COPD: results from COMPERA. Chest. 2021;160:678-89.
261. Dauriat G, Reynaud-Gaubert M, Cottin V, et al. Severe pulmonary hypertension associated with chronic obstructive pulmonary disease: a prospective French multicenter cohort. J Heart Lung Transplant. 2021;40:1009-18.
262. Kovacs G, Agusti A, Barbera JA, et al. Pulmonary vascular involvement in COPD — is there a pulmonary vascular phenotype? Am J Respir Crit Care Med. 2018;198:1000-11.
263. Thabut G, Dauriat G, Stern JB, et al. Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest. 2005;127:1531-6.
264. 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.
265. Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013;158:641-9.
266. Nathan SD, Behr J, Collard HR, et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study. Lancet Respir Med. 2019;7:780-90.
267. Kim NH, Delcroix M, Jais X, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(1):1801915.
268. Валиева З. С., Мартынюк Т. В., Наконечников С. Н., Чазова И. Е. Характеристика пациентов с хронической тромбоэмболической легочной гипертензией по данным российского национального регистра. Терапевтический архив. 2021;93(9):10581065.
269. 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.
270. 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.
271. Wensel R, Francis DP, Meyer FJ, et al. Incremental prognostic value of cardiopulmonary exercise testing and resting haemodynamics in pulmonary arterial hypertension. Int JCardiol. 2013;167:1193-8.
272. 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;41:780-90.
273. Симакова М. А., Злобина И. С., Березина А. В. и др. Эффективность лечения пациентов с хронической тромбоэмболической легочной гипертензией. Кардиология. 2022;62(4):44-54.
274. Пищулов К. А., Симакова М. А., Карпова Д. В., Моисеева О. М. Использование компьютерной томографии в оценке степени тяжести пациентов с хронической тромбоэмболической легочной гипертензией. Артериальная гипертензия. 2021;27(3):333340.
275. Skoro-Sajer N, Marta G, Gerges C, et al. Surgical specimens, haemodynamics and long-term outcomes after pulmonary endarterectomy. Thorax. 2014;69(2):116-22.
276. 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):669-74.
277. Чернявский А. М., Едемский А. Г., Новикова Н. В. и др. Хирургическое лечение хронической тромбоэмболической легочной гипертензии. Национальный медицинский исследовательский центр имени Е. Н. Мешалкина; под общей редакцией А. М. Чернявского. Новосибирск: НМИЦ: Издательство СО РАН, 2019. 317 с.
278. 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;278:232-7.
279. Данилов Н. М., Матчин Ю. Г., Чазова И. Е. Баллонная ангиопластика легочных артерий при неоперабельной хронической тромбоэмболической легочной гипертензии. Сonsilium Medicum. 2016;5:59-61.
280. Марукян Н. В., Симакова М. А., Зубарев Д. Д., Моисеева О. М. Принципы выбора лечебной стратегии у пациентов с неоперабельной формой хронической тромбоэмболической легочной гипертензии. Рациональная фармакотерапия в кардиологии. 2021;17(2):278-85.
281. 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.
282. Lankeit M, Krieg V, Hobohm L, et al. Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2018;37:250-8.
283. Симакова М. А., Гончарова Н. С., Карпова Д. В. и др. Рекомендации по диагностике и лечению пациентов с легочной гипертензией в условиях пандемии COVID-19. Артериальная гипертензия. 2020;26(3):343-55.
284. Акчурин Р. С., Мершин К. В., Табакьян Е. А. и др. Хирургическое лечение хронической тромбоэмболической легочной гипертензии: современные тенденции и собственный опыт. Евразийский Кардиологический Журнал. 2016;2:40-7.
285. D’Armini AM, Morsolini M, Mattiucci G, et al. Pulmonary endarterectomy for distal chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg. 2014;148:1005-11.
286. Taboada D, Pepke-Zaba J, Jenkins DP, et al. Outcome of pulmonary endarterectomy in symptomatic chronic thromboembolic disease. Eur Respir J. 2014;44:1635-45.
287. 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.
288. Чернявский А. М., Едемский А. Г., Чернявский М. А. и др. Пятилетние результаты хирургического лечения пациентов с хронической постэмболической легочной гипертензией. Хирургия. Журнал им. Н. И. Пирогова. 2017;2:21-4.
289. 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.
290. Ghofrani HA, D’Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369:319-29.
291. 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):961-71.
292. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated chronic thromboembolic pulmonary hypertension patients. Eur Respir J. 2018;52:1800248.
293. Benza RL, Farber HW, Frost A, Grunig E, Hoeper MM, Busse D, et al. REVEAL risk score in patients with chronic thromboembolic pulmonary hypertension receiving riociguat. J Heart Lung Transplant. 2018;37:836-43.
294. 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.
295. Bunclark K, Newnham M, Chiu YD, et al. A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension. J Thromb Haemost. 2020;18:114-22.
296. Humbert MS, Simonneau G, Pittrow D, et al. Oral anticoagulants (NOAC and VKA) in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2022;41:716-21.
297. 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;171:685-94.
298. Pengo V, Denas G, Zoppellaro G, et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018;132:1365-71.
299. 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;5:785-94.
300. 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.
301. Reichenberger F, Voswinckel R, Enke B, et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2007;30:922-7.
302. 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.
303. Fukui S, Ogo T, Morita Y, et al. Right ventricular reverse remodelling after balloon pulmonary angioplasty. Eur Respir J. 2014;43:1394-402.
304. Darocha S, Pietura R, Pietrasik A, et al. Improvement in quality of life and hemodynamics in chronic thromboembolic pulmonary hypertension treated with balloon pulmonary angioplasty. Circ J. 2017;81:552-7.
305. Lang I, Meyer BC, Ogo T, et al. Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017;26:160119.
306. Mahmud E, Behnamfar O, Ang L, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Interv Cardiol Clin. 2018;7:103-17.
307. Ogawa A, Matsubara H. After the dawn-balloon pulmonary angioplasty for patients with chronic thromboembolic pulmonary hypertension. Circ J. 2018; 82:1222-30.
308. 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.
309. Данилов Н. М., Матчин Ю. Г., Чернявский А. М. и др. Транслюминальная баллонная ангиопластика легочных артерий у больных с неоперабельной хронической тромбоэмболической легочной гипертензией. Терапевтический архив. 2019;91(4):43-7.
310. Марукян Н. В., Симакова М. А., Карпова Д. В. и др. Реперфузионный отек легкого как осложнение баллонной ангиопластики легочной артерии у пациентов с хронической тромбоэмболической легочной гипертензией. Флебология. 2017;11(4):243-8.
311. Аржанцев А. С., Данилов Н. М., Атанесян Р. В. и др. Перфорация легочных артерий при проведении транслюминальной баллонной ангиопластики. Кардиологический вестник. 2021;16(1):56-63.
312. Данилов Н. М., Яровой С. Ю., Елфимова Е. М. и 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.
313. Brenot P, Jais X, Taniguchi Y, et al. French experience of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53:1802095.
314. 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. doi:10.1161/CIRCULATIONAHA.109.865881.
315. Диагностика и лечение сердечно-сосудистых заболеваний при беременности 2018. Национальные рекомендации. Российский кардиологический журнал. 2018;(3):91-134.
316. 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;39:3165-241.
317. Hemnes AR, Kiely DG, Cockrill BA, et al. Statement on pregnancy in pulmonary hypertension from the Pulmonary Vascular Research Institute. Pulm Circ. 2015;5:435-65.
318. Olsson KM, Channick R. Pregnancy in pulmonary arterial hypertension. Eur Respir Rev. 2016;25:431-7.
319. Condliffe R, Kiely DG. Critical care management of pulmonary hypertension. BJA Education. 2017;17(7):228-34.
320. Gemzell-Danielsson K, Rabe T, Cheng L. Emergency contraception. Gynecol Endocrinol 2013;29:1-14.
321. Roos-Hesselink JW, Budts W, Walker F, et al. Organisation of care for pregnancy in patients with congenital heart disease. Heart. 2017;103:1854-9.
322. Hsu CH, Gomberg-Maitland M, Glassner C, et al. The management of pregnancy and pregnancy-related medical conditions in pulmonary arterial hypertension patients. Int J Clin Pract Suppl. 2011;175:6-14.
323. Terek D, Kayikcioglu M, Kultursay H, et al. Pulmonary arterial hypertension and pregnancy. J Res Med Sci. 2013;18:73-6.
324. 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;39:589-96.
325. 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):362-8.
326. 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.
327. 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):402-9.