Abstract
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.
| Original language | Russian |
|---|---|
| Pages (from-to) | 13-52 |
| Number of pages | 40 |
| Journal | Pulmonologiya |
| Volume | 32 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - 2022 |
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In: Pulmonologiya, Vol. 32, No. 1, 2022, p. 13-52.
Research output: Contribution to journal › Article › Academic › peer-review
TY - JOUR
T1 - Доклад ERS по хронической тромбоэмболической легочной гипертензии
AU - Delcroix, Marion
AU - Torbicki, Adam
AU - Gopalan, Deepa
AU - Sitbon, Olivier
AU - Klok, Frederikus A.
AU - Lang, Irene
AU - Jenkins, David
AU - Kim, Nick H.
AU - Humbert, Marc
AU - Jais, Xavier
AU - Vonk Noordegraaf, Anton
AU - Pepke-Zaba, Joanna
AU - Brénot, Philippe
AU - Dorfmuller, Peter
AU - Fadel, Elie
AU - Ghofrani, Hossein-Ardeschir
AU - Hoeper, Marius M.
AU - Jansa, Pavel
AU - Madani, Michael
AU - Matsubara, Hiromi
AU - Ogo, Takeshi
AU - Grunig, Ekkehard
AU - D'Armini, Andrea
AU - Galie, Nazzareno
AU - Meyer, Bernhard
AU - Corkery, Patrick
AU - Meszaros, Gergely
AU - Mayer, Eckhard
AU - Simonneau, G. rald
N1 - Funding Information: MSD, other (investigator fees received by the institution) from Reata, other (investigator and consultant fees received by the institution) from Bellarophon, other (consultant fees received by the institution) from Acceleron, outside the submitted work. A.Torbicki reports grants and personal fees for lectures and consultancy from Actelion/Janssen, Bayer and MSD, personal fees for lectures from AOP, personal fees for lectures and consultancy from Pfizer, outside the submitted work. D.Gopalan reports other (speaker fees) from Actelion/J&J, other (consultancy work and speaker fees) from Bayer, outside the submitted work. O.Sitbon reports grants, personal fees and non-financial support from Actelion Pharmaceuticals and MSD, grants from GlaxoSmithKline, personal fees from Bayer, Acceleron Pharmaceuticals, Gossamer Bio and Ferrer, outside the submitted work. F.A.Klok reports research grants from Bayer, Bristol-Myers Squibb, Boehringer Ingelheim, Daiichi-Sankyo, MSD and Actelion, the Dutch Heart Foundation and the Dutch Thrombosis association, outside the submitted work. I.Lang reports grants and personal fees from Actelion-Janssen and AOP Orphan Pharma, personal fees from Medtronic, Ferrer and United Therapeutics, outside the submitted work. D.Jenkins reports grants from Bayer, personal fees for advisory board work from Actelion, outside the submitted work. N.H.Kim reports personal fees for consultancy from Actelion, Bayer and Merck, outside the submitted work. M.Humbert reports grants and personal fees from Actelion and Bayer, personal fees from Acceleron, GSK, Merck, Novartis, AstraZeneca and Sanofi, outside the submitted work. X.Jais reports personal fees and non-financial support from Actelion and MSD, grants from Bayer, outside the submitted work. A.Vonk Noordegraaf is supported by the Netherlands CardioVascular Research Initiative (CVON-2012-08 PHAEDRA, CVON-2017-10 DOLPHIN-GENESIS) and the Netherlands Organization for Scientific Research (NWO-VICI: 918.16.610), has received speakers’ money from Johnson & Johnson and Ferrer in the past 3 years, and served as a member of the scientific advisory board of Morphogen-XI. J.Pepke-Zaba has received speaker fees and honoraria for consultations from Actelion, Merck and Bayer, and her institution received research and educational grants from Actelion and Merck. P.Brénot has nothing to disclose. P.Dorfmuller has nothing to disclose. E.Fadel has nothing to disclose. H-A.Ghofrani reports personal fees and other (consultancy fees) from Actelion, Bayer AG, GlaxoSmithKline, Novartis and Pfizer, other (consultancy fees) from Bellerophon Pulse Technologies and MSD, grants from Deutsche Forschungsgemeinschaft (DFG), during the conduct of the study. M.M.Hoeper reports personal fees for consultancy and lectures from Bayer AG, MSD, Actelion, Jansen, Acceleron and Pfizer, during the conduct of the study. P.Jansa reports other (investigator) from Actelion, personal fees and other (investigator) from Bayer Pharma AG and Reata Pharmaceuticals, personal fees from AOP and MSD, outside the submitted work. M.Madani reports personal fees for consultancy from Actelion and Wexler Surgical, outside the submitted work. H.Matsubara reports personal fees from Actelion Pharmaceuticals Japan, Ltd, AOP Orphan Pharmaceuticals AG, Bayer Yakuhin, Ltd, Pfizer Japan, Inc., Nippon Shinyaku, Co., Ltd, Kaneka Medix Corporation, GlaxoSmithKline Pharmaceuticals, Ltd and United Therapeutics Corporation, outside the submitted work. T.Ogo has nothing to disclose. E.Grünig reports fees for lectures and/or consultations from Actelion, Bayer AG, GSK, MSD, United Therapeutics and Pfizer, outside the submitted work. A.D’Armini reports personal fees from Actelion Phamaceuticals, Bayer AG and Merck Sharp & Dohme, outside the submitted work. N.Galie reports grants and personal fees from Actelion and Janssen, personal fees from Pfizer and Ferrer, outside the submitted work. B.Meyer reports personal fees for lectures from Bayer AG, outside the submitted work. P.Corkery has nothing to disclose. G.Meszaros reports personal fees from Actelion Pharmaceuticals, outside the submitted work. E.Mayer reports personal fees for lectures and consultancy from Actelion, Bayer and MSD, during the conduct of the study. G.Simonneau reports personal fees and non-financial support from Actelion, Bayer and MSD, outside the submitted work. Support statement. 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. Acknowledgements. 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. @ERS publications The ERS statement on chronic thromboembolic pulmonary hypertension outlines a review of the literature and current practice concerning diagnosis and management of CTEPH https://bit.ly/34dIjot Received: July 17, 2020. Accepted: November 05, 2020 Copyright © ERS 2021. For reproduction rights and permissions contact [email protected] Disclaimer Acknowledgement. This translated material has not been reviewed prior to release; therefore the European Respiratory Society may not be responsible for any errors, omissions or inaccuracies, or for any consequences arising there from, in the content. Reproduced with permission of the © ERS 2021. European Respiratory Journal. 2021; 57: 2002828. DOI: 10.1183/13993003.02828-2020. Funding Information: Support statement. 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.Humbertis 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. Acknowledgements. 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. Publisher Copyright: Copyright ©ERS 2021.
PY - 2022
Y1 - 2022
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85138151780&partnerID=8YFLogxK
U2 - https://doi.org/10.18093/0869-0189-2022-32-1-13-52
DO - https://doi.org/10.18093/0869-0189-2022-32-1-13-52
M3 - Article
SN - 0869-0189
VL - 32
SP - 13
EP - 52
JO - Pulmonologiya
JF - Pulmonologiya
IS - 1
ER -