Expert consensus on approaches to risk stratification and choice of therapy in patients with inoperable and residual chronic thromboembolic pulmonary hypertension
- Authors: Chazova I.E.1, Martynyuk T.V.1,2, Avdeev S.N.3, Valieva Z.S.1, Vasiltseva O.Y.4, Veselova T.N.1, Gramovich V.V.1, Grankin D.S.4, Danilov N.M.1, Edemskiy A.G.4, Komarov A.L.1, Mershin K.V.1, Moiseeva O.M.5, Chernyavskiy A.M.4, Tsareva N.A.3, Yarovoy S.Y.1
-
Affiliations:
- Chazov National Medical Research Center of Cardiology
- Pirogov Russian National Research Medical University (Pirogov University)
- Sechenov First Moscow State Medical University (Sechenov University)
- Meshalkin National Medical Research Center
- Almazov National Medical Research Centre
- Issue: Vol 97, No 3 (2025): Пульмонология
- Pages: 279-288
- Section: Consensuns
- URL: https://journals.rcsi.science/0040-3660/article/view/291004
- DOI: https://doi.org/10.26442/00403660.2025.03.203237
- ID: 291004
Cite item
Full Text
Abstract
On December 13, 2024, the final working group meeting was held, the purpose of which was to discuss the common position of experts on the problem of chronic thromboembolic pulmonary hypertension of various specialties: cardiologists, cardiac surgeons, pulmonologists, on issues of assessing the operability of patients, risk stratification and choice of therapy in patients with inoperable and residual forms of pathology. The discussion field included a wide range of issues that often arise in real clinical practice, but specific answers to them are not given in national and foreign guidelines. On November 8, 2024, at the preparatory stage, 16 experts under the leadership of Academician of the Russian Academy of Sciences I.E. Chazova (chairman) formulated questions for the preparation of a consensus document (without discussion), consistently discussing the feasibility of including each issue in the document and distributing tasks for the final session. Then, at the intermediate stage, experts searched and systematized scientific information on the formulated questions in the PubMed and eLIBRARY systems to prepare sections of the document in accordance with the established tasks. When finalizing the document, experts answered 28 questions: during anonymous voting an individual score was determined for each block from 1 – completely disagree, to 5 – completely agree. It was considered that a strong consensus was achieved with a mean score of M±m 2.5±2, and with a mean score below 2.5, consensus was not achieved. A strong consensus was reached on all issues.
Full Text
##article.viewOnOriginalSite##About the authors
Irina E. Chazova
Chazov National Medical Research Center of Cardiology
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-9822-4357
(Chairman)
Russian Federation, MoscowTamila V. Martynyuk
Chazov National Medical Research Center of Cardiology; Pirogov Russian National Research Medical University (Pirogov University)
Author for correspondence.
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-9022-8097
(Deputy Chairman)
Russian Federation, Moscow; MoscowSergey N. Avdeev
Sechenov First Moscow State Medical University (Sechenov University)
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-5999-2150
акад. РАН, доктор медицинских наук, профессор, зав. каф. пульмонологии лечебного фак-та, директор Национального медицинского исследовательского центра по профилю «Пульмонология»
Russian Federation, MoscowZarina S. Valieva
Chazov National Medical Research Center of Cardiology
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-9041-3604
доктор медицинских наук, старший научный сотрудник отделения легочной гипертензии и заболеваний сердца
Russian Federation, MoscowOksana Ya. Vasiltseva
Meshalkin National Medical Research Center
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-2932-3159
доктор медицинских наук, вед. науч. сотрудник
Russian Federation, NovosibirskTatiana N. Veselova
Chazov National Medical Research Center of Cardiology
Email: trukhiniv@mail.ru
ORCID iD: 0000-0001-8319-3714
доктор медицинских наук, старший научный сотрудник отделения томографии Института клинической кардиологии им. А.Л. Мясникова
Russian Federation, MoscowVladimir V. Gramovich
Chazov National Medical Research Center of Cardiology
Email: trukhiniv@mail.ru
ORCID iD: 0000-0003-3292-0912
кандидат медицинских наук, старший научный сотрудник отделения легочной гипертензии и заболеваний сердца Института клинической кардиологии им. А.Л. Мясникова
Russian Federation, MoscowDenis S. Grankin
Meshalkin National Medical Research Center
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-0083-8888
кандидат медицинских наук, врач по рентгенэндоваскулярной диагностике и лечению
Russian Federation, NovosibirskNikolay M. Danilov
Chazov National Medical Research Center of Cardiology
Email: trukhiniv@mail.ru
ORCID iD: 0000-0001-9853-9087
доктор медицинских наук, ведущий научный сотрудник отд. гипертонии Института клинической кардиологии им. А.Л. Мясникова
Russian Federation, MoscowAlexander G. Edemskiy
Meshalkin National Medical Research Center
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-6661-7826
кандидат медицинских наук, врач – сердечно-сосудистый хирург
Russian Federation, NovosibirskAndrey L. Komarov
Chazov National Medical Research Center of Cardiology
Email: trukhiniv@mail.ru
ORCID iD: 0000-0001-9141-103X
доктор медицинских наук, ведущий научный сотрудник отд. клинических проблем атеротромбоза
Russian Federation, MoscowKirill V. Mershin
Chazov National Medical Research Center of Cardiology
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-7256-0563
кандидат медицинских наук, сердечно-сосудистый хирург 4-го кардиохирургического отд-ния
Russian Federation, MoscowOlga M. Moiseeva
Almazov National Medical Research Centre
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-7817-3847
доктор медицинских наук, профессор, рук., гл. научный сотрудник отд. некоронарогенных заболеваний сердца, директор Института сердца и сосудов, врач-кардиолог
Russian Federation, Saint PetersburgAleksandr M. Chernyavskiy
Meshalkin National Medical Research Center
Email: trukhiniv@mail.ru
ORCID iD: 0000-0001-9818-8678
чл.-кор. РАН, доктор медицинских наук, профессор, ген. директор
Russian Federation, NovosibirskNatalia A. Tsareva
Sechenov First Moscow State Medical University (Sechenov University)
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-5448-9222
кандидат медицинских наук, доц. каф. Пульмонологии
Russian Federation, MoscowSergey Yu. Yarovoy
Chazov National Medical Research Center of Cardiology
Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-1982-3061
кандидат медицинских наук, мл. научный сотрудник отд. гипертонии Научно-исследовательского института клинической кардиологии им. А.Л. Мясникова
Russian Federation, MoscowReferences
- 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 [Chazova IE, Martynyuk TV, Valieva ZS, et al. Eurasian Association of Cardiology (EAC) guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (2020). Eurasian Heart Journal. 2021;(1):6-43 (in Russian)]. doi: 10.38109/2225-1685-2021-1-6-43
- Чазова И.Е., Мартынюк Т.В., Шмальц А.А., и др. Евразийские рекомендации по диагностике и лечению легочной гипертензии (2023). Евразийский Кардиологический Журнал. 2024;(1):6-85 [Chazova IE, Martynyuk TV, Shmalts AA, et al. Eurasian guidelines for the diagnosis and treatment of pulmonary hypertension (2023). Eurasian Heart Journal. 2024;(1):6-85 (in Russian)]. doi: 10.38109/2225-1685-2024-1-6-85
- Авдеев С.Н., Барбараш О.Л., Валиева З.С., и др. Легочная гипертензия, в том числе хроническая тромбоэмболическая легочная гипертензия. Клинические рекомендации 2024. Российский кардиологический журнал. 2024;29(11):6161 [Avdeev SN, Barbarash OL, Valieva ZS, et al. 2024 Clinical practice guidelines for Pulmonary hypertension, including chronic thromboembolic pulmonary hypertension. Russian Journal of Cardiology. 2024;29(11):6161 (in Russian)]. doi: 10.15829/1560-4071-2024-6161
- Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023;61(1):2200879. doi: 10.1183/13993003.00879-2022
- Hoeper MM. Residual Pulmonary Hypertension After Pulmonary Endarterectomy: The Fog Is Clearing. Circulation. 2016;133(18):1731-3. doi: 10.1161/CIRCULATIONAHA.116.022595
- 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(1):97-103; discussion 103. doi: 10.1016/j.athoracsur.2012.04.004
- Mayer E, Jenkins D, Lindner J, et al. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011;141(3):702-10. doi: 10.1016/j.jtcvs.2010.11.024
- 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 [Akchurin RS, Mershin KV, Tabakyan EA, et al. Surgical treatment of chronic thromboembolic pulmonary hypertension: modern trends and own experience. Eurasian Heart Journal. 2016;(2):40-7 (in Russian)]. doi: 10.38109/2225-1685-2016-2-40-47
- D'Armini AM, Morsolini M, Mattiucci G, et al. Pulmonary endarterectomy for distal chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg. 2014;148(3):1005-11;1012.e1-2;discussion 1011-2. doi: 10.1016/j.jtcvs.2014.06.052
- Акчурин Р.С., Мершин К.В., Лепилин М.Г., Табакьян Е.А. Операции тромбэндартерэктомии из легочной артерии: состояние проблемы. Consilium Medicum. 2016;18(5):62-6 [Akchurin RS, Mershin KV, Lepilin MG, Tabakian EA. Pulmonary thromboendarterectomy: state of the problem. Consilium Medicum. 2016;18(5):62-6 (in Russian)].
- Данилов Н.М., Матчин Ю.Г., Чазова И.Е. Баллонная ангиопластика легочных артерий при неоперабельной хронической тромбоэмболической легочной гипертензии. Consilium Medicum. 2016;18(5):59-61 [Danilov NM, Matchin YuG, Chazova IE. Balloon pulmonary angioplasty in patient with inoperable chronic thromboembolic pulmonary hypertension. Consilium Medicum. 2016;18(5):59-61 (in Russian)].
- Delcroix M, Pepke-Zaba J, D'Armini AM, et al. Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy. Circulation. 2024;150(17):1354-35. doi: 10.1161/CIRCULATIONAHA.124.068610
- 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;278:232-3. doi: 10.1016/j.ijcard.2018.10.066
- 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 [Marukyan NV, Simakova MA, Zubarev DD, Moiseeva OM. Principles of choosing a treatment strategy in patients with inoperable chronic thromboembolic pulmonary hypertension. Rational Pharmacotherapy in Cardiology. 2021;17(2):278-85 (in Russian)]. doi: 10.20996/1819-6446-2021-04-02
- 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 [Danilov NM, Matchin YG, Chernyavsky AM, et al. Balloon pulmonary angioplasty for patients with inoperable chronic thromboembolic pulmonary hypertension. Terapevticheskii Arkhiv (Ter. Arkh.). 2019;91(4):43-7 (in Russian)]. doi: 10.26442/00403660.2019.04.000100
- Хирургическое лечение хронической тромбоэмболической легочной гипертензии. Под ред. А.М. Чернявского. Новосибирск: Изд-во СО РАН, 2019 [Khirurgicheskoe lechenie khronicheskoi tromboembolicheskoi legochnoi gipertenzii. Pod red. AM Cherniavskogo. Novosibirsk: Izd-vo SO RAN, 2019 (in Russian)].
- Jenkins D, Madani M, Fadel E, et al. Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017;26(143):160111. doi: 10.1183/16000617.0111-2016
- Хроническая тромбоэмболическая легочная гипертензия. Руководство для врачей. Под ред. Т.В. Мартынюк, акад. РАН И.Е. Чазовой. М.: Медицинское информационное агентство, 2023 [Khronicheskaia tromboembolicheskaia legochnaia gipertenziia. Rukovodstvo dlia vrachei. Pod red. TV Martyniuk, akad. RAN IE Chazovoi. Moscow: Meditsinskoe informatsionnoe agentstvo, 2023 (in Russian)].
- Kim NH, D'Armini AM, Delcroix M, et al. Chronic thromboembolic pulmonary disease. Eur Respir J. 2024;64(4). doi: 10.1183/13993003.01294-2024
- Чазова И.Е., Мартынюк Т.В., Филиппов Е.В., и др. Клинические рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (II часть). Терапевтический архив. 2016;88(10):63-74 [Chazova IE, Martynyuk TV, Filippov EV, et al. Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2). Terapevticheskii Arkhiv (Ter. Arkh.). 2016;88(10):63-74 (in Russian)]. doi: 10.17116/terarkh201688663-73
- Delcroix M, Torbicki A, Gopalan D, et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J. 2021;57(6):2002828. doi: 10.1183/13993003.02828-2020
- 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. Российский электронный журнал лучевой диагностики. 2022;12(2):94-103 [Veselova TN, Demchenkova AYu, En MYu, et al. The assessment of lung perfusion in patients with chronic thromboembolic pulmonary hypertension using CT and scintigraphy. Russian Electronic Journal of Radiology. 2022;12(2):94-103 (in Russian)]. doi: 10.21569/2222-7415-2022-12-2-94-103
- Чазова И.Е., Мартынюк Т.В. Клинические рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (I часть). Терапевтический архив. 2016;88(9):90-101 [Chazova IE, Martynyuk TV. Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1). Terapevticheskii Arkhiv (Ter. Arkh.). 2016;88(9):90-101 (in Russian)]. doi: 10.17116/terarkh201688990-101
- Gopalan D, Delcroix M, Held M. Diagnosis of chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017;26(143):160108. doi: 10.1183/16000617.0108-2016
- Wilkens H, Konstantinides S, Lang IM, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): Updated Recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018;272S:69-78. doi: 10.1016/j.ijcard.2018.08.079
- Cannon JE, Su L, Kiely DG, et al. Dynamic Risk Stratification of Patient Long-Term Outcome After Pulmonary Endarterectomy: Results From the United Kingdom National Cohort. Circulation. 2016;133(18):1761-71. doi: 10.1161/CIRCULATIONAHA.115.019470
- 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 [Valieva ZS, Martynyuk TV. Choice of specific and anticoagulant therapy in patients with newly diagnosed chronic thromboembolic pulmonary hypertension depending on the operability status. Russian Journal of Cardiology. 2023;28(3):5231 (in Russian)]. doi: 10.15829/1560-4071-2023-5231
- Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated chronic thromboembolic pulmonary hypertension patients. Eur Respir J. 2018;52(5):1800248. doi: 10.1183/13993003.00248-2018
- 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. doi: 10.1093/eurheartj/ehv317
- Pulmonary Embolism & Pulmonary Vascular Diseases Group of the Chinese Thoracic Society, Pulmonary Embolism & Pulmonary Vascular Disease Working Group of Chinese Association of Chest Physicians, National Cooperation Group on Prevention & Treatment of Pulmonary Embolism & Pulmonary Vascular Disease, National Expert Panel on the Development of a Standardized Framework for Pulmonary Arterial Hypertension. Guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (2024 edition). Zhonghua Yi Xue Za Zhi. 2024;104(24):2200-21 (in Chinese). doi: 10.3760/cma.j.cn112137-20240116-00117
- Ulrich S, Saxer S, Hasler ED, et al. Effect of domiciliary oxygen therapy on exercise capacity and quality of life in patients with pulmonary arterial or chronic thromboembolic pulmonary hypertension: a randomised, placebo-controlled trial. Eur Respir J. 2019;54(2). doi: 10.1183/13993003.002762019
- Елфимова Е.М., Данилов Н.М., Яровой С.Ю., и др. Влияние СРАР-терапии на развитие реперфузионных поражений легких после транслюминальной баллонной ангиопластики легочных артерий у пациентов с хронической тромбоэмболической легочной гипертензией. Системные гипертензии. 2023;20(3):11-8 [Elfimova EM, Danilov NM, Yarovoy SY, et al. Effect of CPAP therapy on the reperfusion lung injury after transluminal balloon angioplasty of the pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension. Systemic Hypertension. 2023;20(3):11-8 (in Russian)]. doi: 10.38109/2075-082x-2023-3-11-18
- 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(Suppl. K):K46-53. doi: 10.1093/eurheartj/suz207
- Klok FA, Ageno W, Ay C, et al. Optimal follow-up after acute pulmonary embolism: a position paper of the European Society of Cardiology Working Group on Pulmonary Circulation and Right Ventricular Function, in collaboration with the European Society of Cardiology Working Group on Atherosclerosis and Vascular Biology, endorsed by the European Respiratory Society. Eur Heart J. 2022;43(3):183-8. doi: 10.1093/eurheartj/ehab816
- 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(4):543-603. doi: 10.1093/eurheartj/ehz405
- Delcroix M, Lang I, Pepke-Zaba J, et al. Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry. Circulation. 2016;133(9):859-71. doi: 10.1161/CIRCULATIONAHA.115.016522
- Corsico AG, D'Armini AM, Cerveri I, et al. Long-term outcome after pulmonary endarterectomy. Am J Respir Crit Care Med. 2008;178(4):419-24. doi: 10.1164/rccm.200801-101OC
- Madani MM, Wiedenroth CB, Jenkins DP, et al. Pulmonary Thromboendarterectomy: The Potentially Curative Treatment of Choice for Chronic Thromboembolic Pulmonary Hypertension. Ann Thorac Surg. 2025;119(4):756-67. doi: 10.1016/j.athoracsur.2024.07.052
- Ghofrani HA, D'Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369(4):319-29. doi: 10.1056/NEJMoa1209657
- Simonneau G, D'Armini AM, Ghofrani HA, et al. Predictors of long-term outcomes in patients treated with riociguat for chronic thromboembolic pulmonary hypertension: data from the CHEST-2 open-label, randomised, long-term extension trial. Lancet Respir Med. 2016;4(5):372-80. doi: 10.1016/S2213-2600(16)30022-4
- Ghofrani HA, Gomez Sanchez MA, Humbert M, et al. Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry. Respir Med. 2021;178:106220. doi: 10.1016/j.rmed.2020.106220
- van Thor MCJ, Ten Klooster L, Snijder RJ, et al. Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension. Int J Cardiol Heart Vasc. 2019;22:163-6. doi: 10.1016/j.ijcha.2019.02.004
- Mehta S, Helmersen D, Provencher S, et al. Diagnostic evaluation and management of chronic thromboembolic pulmonary hypertension: a clinical practice guideline. Can Respir J. 2010;17(6):301-34. doi: 10.1155/2010/704258
- Цыренов Д.Д., Акчурин Р.С., Мершин К.В., и др. Кардиологические аспекты периоперационного ведения больных хронической тромбоэмболической легочной гипертензией при тромбэндартерэктомии из ветвей легочной артерии. Евразийский Кардиологический Журнал. 2021;(1):94-104 [Tsyrenov DD, Akchurin RS, Mershin KV, et al. Cardiological aspects of perioperative management of patients with chronic thromboembolic pulmonary hypertension during thromboendarterectomy from pulmonary artery branches. Eurasian Heart Journal. 2021;(1):94-104 (in Russian)]. doi: 10.38109/2225-1685-2021-1-94-104
Supplementary files
