Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. It is caused by persistent obstruction of pulmonary arteries by chronic organised fibrotic clots, despite adequate anticoagulation. The pulmonary hypertension is also caused by concomitant microvasculopathy which may progress without timely treatment. Timely and accurate diagnosis requires the combination of imaging and haemodynamic assessment. Optimal therapy should be individualised to each case and determined by an experienced multidisciplinary CTEPH team with the ability to offer all current treatment modalities. This report summarises current knowledge and presents key messages from the International CTEPH Conference, Bad Nauheim, Germany, 2021. Sessions were dedicated to 1) disease definition; 2) pathophysiology, including the impact of the hypertrophied bronchial circulation, right ventricle (dys)function, genetics and inflammation; 3) diagnosis, early after acute pulmonary embolism, using computed tomography and perfusion techniques, and supporting the selection of appropriate therapies; 4) surgical treatment, pulmonary endarterectomy for proximal and distal disease, and peri-operative management; 5) percutaneous approach or balloon pulmonary angioplasty, techniques and complications; and 6) medical treatment, including anticoagulation and pulmonary hypertension drugs, and in combination with interventional treatments. Chronic thromboembolic pulmonary disease without pulmonary hypertension is also discussed in terms of its diagnostic and therapeutic aspects.
Competing Interests: Conflict of interest: E. Fadel, G. Martinez, G. Simonneau, K. Salaunkey, N. Screaton and P. Brénot have no conflicts of interest to declare. 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); his institute received speakers fees from Johnson & Johnson, MSD, Actelion, Bayer and Ferrer in the past 3 years; and he served as a member of the scientific advisory boards of Morphogen-X, Ferrer, Gossamer Bio Services Inc. and Johnson & Johnson. C.B. Wiedenroth has received speaker fees and/or consultant honoraria from Actelion, AOP Orphan Pharmaceuticals AG, Bayer AG, BTG, MSD and Pfizer. D.P. Jenkins is on the adjudication committees for the SELECT and Maciteph studies (Actelion), and has received fees for lectures on CTEPH from Actelion. E. Mayer has received speaker or consulting fees from Actelion/Janssen, Bayer and MSD. F.A. Klok has received research support from Bayer, Bristol-Myers Squibb, Boehringer Ingelheim, MSD, Leo Pharma, Actelion, the Netherlands Organisation for Health Research and Development, the Dutch Thrombosis Association, the Dutch Heart Foundation and the Horizon Europe Program, unrelated to the current work and paid to his institution. H.A. Ghofrani has received grants/research support from Actelion, Janssen, Bayer, Gossamer, Acceleron; honoraria or consultation fees from Actelion, Janssen, Bayer, Gossamer, Acceleron, Pfizer, Novartis and MSD; and participated in a company sponsored speaker's bureau for Actelion, Janssen, Bayer, Gossamer, Pfizer, Novartis and MSD. H. Matsubara has received honoraria for lectures from Janssen Pharmaceutical KK, Bayer Yakuhin Ltd, Nippon Shinyaku Co Ltd, Mochida Pharmaceutical Co. Ltd and Kaneka Medix Corporation. I.M. Lang has a financial interest with Actelion/Janssen, AOP Orphan, Medtronic, Daiichi Sankyo, Neutrolis, United Therapeutics and Ferrer that could be perceived as a real or apparent conflict of interest in the context of the subject of this article. J. Pepke-Zaba has received speaker or consulting fees from Actelion/Janssen, Bayer, MSD and Ferrer, and a research grant from MSD, all outside submitted work. M.C. McInnis reports honoraria from Bayer and Boehringer Ingelheim, unrelated to the current work. M. Delcroix reports research grants from Actelion/Janssen, speaker and consultant fees from Altavant, Acceleron, AOP, Daiichi Sankyo, Bayer, Ferrer, Gossamer and MSD, outside the submitted work, and all paid to her institution. She is holder of the Janssen Chair for Pulmonary Hypertension at KU Leuven. M.M. Hoeper reports fees for lectures and/or consultations from Acceleron, Actelion, Bayer, GSK, Janssen, MSD and Pfizer. M.M. Madani is a consultant for Actelion. M. Toshner reports grants and personal fees from Bayer, personal fees from MSD, grants and personal fees from Actelion, and personal fees from GSK within the last 5 years. N.H. Kim has served as consultant for Bayer, Janssen, Merck and United Therapeutics. S. Guth has received speaker fees and/or consultant honoraria from Actelion, Bayer, GSK, MSD and Pfizer. X. Jaïs reports grants from the French Ministry of Health and Bayer Healthcare, during the conduct of the study; grants from Acceleron, Janssen and MSD; personal fees from Janssen and MSD; and non-financial support from Janssen.
(Copyright ©The authors 2023.)