References

pulmo

Пульмонология

PULMONOLOGIYA

0869-01892541-9617

Scientific and Practical Journal “PULMONOLOGIYA” LLC

10.18093/0869-0189-2022-32-1-13-52

pulmo-3503

Research Article

КЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИ

CLINICAL GUIDELINES

Доклад ERS по хронической тромбоэмболической легочной гипертензии

ERS statement on chronic thromboembolic pulmonary hypertension

https://orcid.org/0000-0001-8394-9809

Делькруа

М.

Delcroix

М.

Марион Делькруа, Сопредседатель

Херестраат 49, 3000 Левен

Marion Delcroix. Co-chair

Herestraat 49, 3000 Leuven

marion.delcroix@uzleuven.be

https://orcid.org/0000-0003-3475-8832

Торбицки

А.

Torbicki

А.

Адам Торбицки. Редакторы разделов

Отцвок

Adam Torbicki. Section editors

Otwock

Гопалан

Д.

Gopalan

Дипа Гопалан. Редакторы разделов

Лондон

Deepa Gopalan. Section editors

London

https://orcid.org/0000-0002-1942-1951

Ситбон

О.

Sitbon

Оливье Ситбон. Редакторы разделов

Ле Кремлен-Бисетр

Olivier Sitbon. Section editors

Le Kremlin-Bicêtre

https://orcid.org/0000-0001-9961-0754

Клок

Ф. А.

Klok

F. A.

Фредерикус А. Клок. Редакторы разделов

Лейден

Frederikus A. Klok. Section editors

Leiden

Ланг

И.

Lang

Ирен Ланг. Редакторы разделов

Вена

Irene Lang. Section editors

Vienna

Дженкинс

Д.

Jenkins

Дэвид Дженкинс. Редакторы разделов

Кембридж

David Jenkins. Section editors

Cambridge

Ким

Н. Х.

Kim

N. H.

Ник Х. Ким. Редакторы разделов

Ла-Хойя, Калифорния

Nick H. Kim. Section editors

La Jolla, CA

https://orcid.org/0000-0003-0703-2892

Умбер

М.

Humbert

Марк Умбер. Редакторы разделов

Ле Кремлен-Бисетр

Marc Humbert. Section editors

Le Kremlin-Bicêtre

Джайс

К.

Jais

Ксавье Джайс. Редакторы разделов

Ле Кремлен-Бисетр

Xavier Jais. Section editors

Le Kremlin-Bicêtre

https://orcid.org/0000-0002-4057-758X

Нордеграаф

Антон Ф.

Noordegraaf

A. V.

Антон Фонк Нордеграаф. Редакторы разделов

Амстердам

Anton Vonk Noordegraaf. Section editors

Amsterdam

Пепке-Заба

Д.

Pepke-Zaba

Джоанна Пепке-Заба. Редакторы разделов

Кембридж

Joanna Pepke-Zaba. Section editors

Cambridge

Брено

Ф.

Brénot

Филипп Брено

Ле-Плесси-Робинсон

Philippe Brénot

Le Plessis Robinson

Дорфмюллер

П.

Dorfmuller

Петер Дорфмюллер

Гисен

Лондон

Бад-Наухайм

Peter Dorfmuller

Giessen

London

Bad Nauheim

Фадель

Э.

Fadel

Эли Фадель

Ганновер

Elie Fadel

Hannover

https://orcid.org/0000-0002-2029-4419

Гофрани

Х.-А.

Ghofrani

H.-A.

Хоссейн-Ардешир Гофрани

Гисен

Лондон

Бад-Наухайм

Hossein-Ardeschir Ghofrani

Giessen

London

Bad Nauheim

https://orcid.org/0000-0001-9086-2293

Хупер

Мариус М.

Hoeper

M. M.

Мариус М. Хупер

Ле Кремлен-Бис

Ганновер

Marius M. Hoeper

Le Kremlin-Bicêtre

Hannover

Янса

П.

Jansa

Павел Янса

Прага

Pavel Jansa

Prague

Мадани

М.

Madani

Майкл Мадани

Сан-Диего, Калифорния

Michael Madani

San Diego

https://orcid.org/0000-0002-3417-7651

Мацубара

Х.

Matsubara

Хироми Мацубара

Окаяма

Hiromi Matsubara

Okayama

Ого

Т.

Ogo

Такеши Ого

Осака

Takeshi Ogo

Osaka

Д’Армини

А.

D’Armini

Андреа Д’Армини

Павия

Andrea D’Armini

Pavia

Галие

Н.

Galie

Назарено Галие

Болонья

Nazzareno Galie

Bologna

Мейер

Б.

Meyer

Бернхард Мейер

Ганновер

Bernhard Meyer

Hannover

Коркери

П.

Corkery

Патрик Коркери. Представитель пациентов

Дублин

Patrick Corkery. Patient representative

Dublin

Месарош

Г.

Meszaros

Гергели Месарош. Представитель пациентов

Gergely Meszaros. Patient representative

Майер

Э.

Mayer

Экхард Майер. Равный вклад. Сопредседатель

Бад-Наухайм

Eckhard Mayer. Equal contribution. Co-chair

Bad Nauheim

Симонно

Ж.

Simonneau

Жеральд Симонно. Равный вклад. Сопредседатель

Ле Кремлен-Бисетр

Gérald Simonneau. Equal contribution. Co-chair

Le Kremlin-Bicêtre

Клиническое отделение респираторных заболеваний, Центр легочной гипертензии, Левенская университетская больница; Лаборатория респираторных заболеваний и торакальной хирургии (BREATHE), кафедра хронических болезней и метаболизма (CHROMETA), Левенский католический университет;БельгияClinical Dept of Respiratory Diseases, Pulmonary Hypertension Center; BREATHE, Dept CHROMETA, KU LeuvenBelgium

Отделение легочного кровообращения, тромбоэмболические заболевания и кардиология, центр последипломного медицинского образования, Европейский центр здоровьяПольшаDept of Pulmonary Circulation, Thrombo-embolic Diseases and Cardiology, Center of Postgraduate Medical Education, ECZ-OtwockPoland

Отделение лучевой диагностики, Имперский колледж, больницы Национальной системы здравоохранения ВеликобританииВеликобританияDept of Radiology, Imperial College Hospitals NHS TrustsUnited Kingdom

Университет Париж-Сакле; отделение Inserm UMR_S 999, пульмонологическая служба, больница Бисетр (AP-HP)ФранцияUniversité Paris-Saclay; Inserm UMR_S 999, Service de Pneumologie, Hôpital Bicêtre (AP-HP)France

Кафедра медицины, отделение тромбоза и гемостаза, Медицинский центр Лейденского университетаНидерландыDept of Medicine – Thrombosis and Hemostasis, Leiden University Medical CenterNetherlands

Венский медицинский университетАвстрияMedical University of ViennaAustria

Королевская больница Папворта, больница Кембриджского университетаВеликобританияRoyal Papworth Hospital, Cambridge University HospitalUnited Kingdom

Отделение пульмонологии, реанимации и медицины сна, Калифорнийский университет в Сан-ДиегоСоединённые Штаты АмерикиDivision of Pulmonary, Critical Care, and Sleep Medicine, University of California San DiegoUnited States

Кафедра легочной медицины, Амстердамский медицинский центр, Амстердамский свободный университет, Центр исследований сердечно-сосудистой системыНидерландыDept of Pulmonary Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam Cardiovascular SciencesNetherlands

Больница Мари Ланнелонг, Университет Париж-ЮгФранцияMarie Lannelongue Hospital, Paris-South UniversityFrance

Гисенский университет и Марбургский пульмонологический центр, Немецкий центр исследований легких (DZL); Медицинская кафедра, Имперский колледж Лондона; Отделение пульмонологии, Клиника КеркхоффГерманияUniversity of Giessen and Marburg Lung Center, German Center of Lung Research (DZL); Dept of Medicine, Imperial College London; Dept of Pneumology, Kerckhoff-Clinic Bad NauheimGermany

Высшая медицинская школа ГанновераGermanyHannover Medical SchoolGermany

Университет Париж-Сакле; отделение Inserm UMR_S 999, пульмонологическая служба, больница Бисетр (AP-HP); Высшая медицинская школа ГанновераФранцияUniversité Paris-Saclay; Inserm UMR_S 999, Service de Pneumologie, Hôpital Bicêtre (AP-HP); Hannover Medical SchoolFrance

2-е медицинское отделение, кафедра сердечно-сосудистой медицины, Первый медицинский факультет, Карлов университет и общая университетская больницЧехия2nd Department of Medicine, Dept of Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital in PragueCzech Republic

Сердечно-сосудистый центр Сульпицио, Калифорнийский университетСоединённые Штаты АмерикиSulpizio Cardiovascular Centre, University of CaliforniaUnited States

Национальная больничная организация Медицинский центр ОкаямаЯпонияNational Hospital Organization Okayama Medical CenterJapan

Национальный церебральный и сердечно-сосудистый центрЯпонияNational Cerebral and Cardiovascular CentreJapan

Отделение кардиохирургии, интраторакальной трансплантации и легочной гипертензии, Медицинский факультет Университета Павии, Поликлиника научно-исследовательского центра Фонда Сан-МаттеоИталияUnit of Cardiac Surgery, Intrathoracic Transplantation and Pulmonary Hypertension, University of Pavia School of Medicine, Foundation I.R.C.C.S. Policlinico San MatteoItaly

Университет БолоньиИталияUniversity of BolognaItaly

Институт диагностической и интервенционной радиологии, Высшая медицинская школа ГанновераГерманияInstitute for Diagnostic and Interventional Radiology, Hannover Medical SchoolGermany

Ирландское отделение Ассоциации легочной гипертензииИрландияPHA IrelandIreland

Европейское отделение Ассоциации легочной гипертензииВенгрияPHA EuropeHungary

Отделение торакальной хирургии, Клиника КеркхоффГерманияDept of Thoracic Surgery, Kerckhoff Clinic Bad NauheimGermany

2022

21022022

3211352

Copyright © Делькруа М., Торбицки А., Гопалан Д., Ситбон О., Клок Ф.А., Ланг И., Дженкинс Д., Ким Н.Х., Умбер М., Джайс К., Нордеграаф А., Пепке-Заба Д., Брено Ф., Дорфмюллер П., Фадель Э., Гофрани Х., Хупер М., Янса П., Мадани М., Мацубара Х., Ого Т., Д’Армини А., Галие Н., Мейер Б., Коркери П., Месарош Г., Майер Э., Симонно Ж., 2022

2022

Делькруа М., Торбицки А., Гопалан Д., Ситбон О., Клок Ф.А., Ланг И., Дженкинс Д., Ким Н.Х., Умбер М., Джайс К., Нордеграаф А., Пепке-Заба Д., Брено Ф., Дорфмюллер П., Фадель Э., Гофрани Х., Хупер М., Янса П., Мадани М., Мацубара Х., Ого Т., Д’Армини А., Галие Н., Мейер Б., Коркери П., Месарош Г., Майер Э., Симонно Ж.

Delcroix М., Torbicki А., Gopalan D., Sitbon O., Klok F., Lang I., Jenkins D., Kim N., Humbert M., Jais X., Noordegraaf A., Pepke-Zaba J., Brénot P., Dorfmuller P., Fadel E., Ghofrani H., Hoeper M., Jansa P., Madani M., Matsubara H., Ogo T., D’Armini A., Galie N., Meyer B., Corkery P., Meszaros G., Mayer E., Simonneau G.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://journal.pulmonology.ru/pulm/article/view/3503

Хроническая тромбоэмболическая (ХТЭ) легочная гипертензия (ЛГ) – редкое осложнение острой тромбоэмболии легочной артерии (ТЭЛА) – как симптоматической, так и бессимптомной. При окклюзии проксимальных сегментов легочных артерий фиброзным внутрисосудистым материалом в комбинации со вторичной микроваскулопатией сосудов диаметром < 500 мкм повышаются легочное сосудистое сопротивление и прогрессирующая правожелудочковая недостаточность. Механизм трансформации кровяных сгустков в остаточный фиброзный материал до сих пор не установлен. ХТЭ ЛГ у пациентов с ЛГ подозревается в случае, когда по данным вентиляционно-перфузионной сцинтиграфии легких выявляются несоответствующие картине вентиляции дефекты перфузии, которые подтверждаются при катетеризации правых отделов сердца и визуализации сосудов. В зависимости от локализации и типов повреждений, помимо пожизненного приема антикоагулянтов, на сегодняшний день доступны хирургическое лечение, ангиопластика и медикаментозное лечение. В настоящем докладе представлен обзор литературы и описана современная практика установления диагноза и ведения больных ХТЭ ЛГ. Документ охватывает области определения, диагностики, эпидемиологии, наблюдения после острой ТЭЛА, патофизиологии, лечения с помощью легочной тромбэндартерэктомии, баллонной ангиопластики легочной артерии, лекарственных препаратов и их комбинаций, реабилитации и новых направлений исследований в области ХТЭ ЛГ. Доклад представляет собой результат первого совместного проекта по ЛГ Европейского респираторного общества, Международной ассоциации хронической тромбоэмболической легочной гипертензии и Европейской сети медицинских учреждений по редким заболеваниям легких. В докладе резюмируются текущие знания, но не даются формальные рекомендации для клинической практики.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with a secondary microvasculopathy of vessels < 500 μm, leads to increased pulmonary vascular resistance and progressive right heart failure. The mechanism responsible for the transformation of red clots into fibrotic material remnants has not yet been elucidated. In patients with pulmonary hypertension, the diagnosis is suspected when a ventilation/ perfusion lung scan shows mismatched perfusion defects, and confirmed by right heart catheterisation and vascular imaging. Today, in addition to lifelong anticoagulation, treatment modalities include surgery, angioplasty and medical treatment according to the localisation and characteristics of the lesions. This statement outlines a review of the literature and current practice concerning diagnosis and management of CTEPH. It covers the definitions, diagnosis, epidemiology, follow-up after acute pulmonary embolism, pathophysiology, treatment by pulmonary endarterectomy, balloon pulmonary angioplasty, drugs and their combination, rehabilitation and new lines of research in CTEPH. It represents the first collaboration of the European Respiratory Society, the International CTEPH Association and the European Reference Network-Lung in the pulmonary hypertension domain. The statement summarises current knowledge, but does not make formal recommendations for clinical practice.

Международная ассоциация ХТЭ ЛГ (ICA) компенсировала транспортные расходы членам ее совета М.Делькруа, Э.Фадель, Д.Дженкинс, Н.Х.Ким, И.Ланг, M.Maдани, Э.Майер, Х.Мацубара, Д.Пепке-Заба и Ж.Симонно. M.Умбер получал финансирование в рамках программы Investissement d’Avenir programme Французского национального агентства по научным исследованиям по гранту № ANR-18-RHUS-0006 (DESTINATION 2024). Европейское респираторное общество поддержало проект грантом № TF 2018-04. Сведения о финансировании в рамках подготовки настоящей статьи были переданы в реестр финансирования Crossref Funder Registry. Благодарим Thomy Tonia, старшего методолога ERS, за обзор методологии, и Valérie Vaccaro, руководителя проекта ERS по научной деятельности, за организацию заседаний рабочей группы. Выражаем благодарность Patrick Corkery из Ирландского отделения Ассоциации легочной гипертензии и Gergely Meszaros из Европейского отделения Ассоциации легочной гипертензии, которые предоставили информацию о взглядах и предпочтениях пациентов в ходе телеконференций, личных встреч и в письменной форме. Г-н P.Corkery и г-н G.Meszaros внесли свой вклад в формулировку и приоритезацию ключевых вопросов, выявление аспектов, важных для пациентов, и в рецензировании окончательной рукописи. Patrick Corkery перенес легочную тромбэндартерэктомию в Королевской больнице Папворта в январе 2016 г., выздоровел и переплыл Ла-Манш в сентябре 2017 г.

Travel support was provided by the International CTEPH Association (ICA) for its board members: M.Delcroix, E.Fadel, D.Jenkins, N.Kim, I.Lang, M.Madani, E.Mayer, H.Matsubara, J.Pepke-Zaba, and G.Simonneau. M.Humbert is supported by the Investissement d’Avenir programme managed by the French National Research Agency under the grant contract ANR-18-RHUS-0006 (DESTINATION 2024). The project was supported by European Respiratory Society grant TF 2018-04. Funding information for this article has been deposited with the Crossref Funder Registry. We thank Thomy Tonia, ERS Senior Methodologist, for her methodological overview and Valérie Vaccaro, ERS Scientific Activities Project Lead, for organising the task force meetings. With thanks to Patrick Corkery, PHA Ireland and Gergely Meszaros, PHA Europe, who provided input on patient views and preferences via teleconferences, attendance at face to face meetings, and in writing. Mr Corkery and Mr Meszaros contributed to formulating and prioritising the key questions, identifying aspects important to patients, and in reviewing the final manuscript. Patrick Corkery underwent PEA surgery in Royal Papworth Hospital in January 2016, recovered and swam the English Channel in September 2017.

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The authors declare that there are no conflicts of interest present.