TY - JOUR T1 - Current strategies for managing CTEPH: results of the worldwide prospective CTEPH Registry JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00850-2020 SP - 00850-2020 AU - Stefan Guth AU - Andrea M. D'Armini AU - Marion Delcroix AU - Kazuhiko Nakayama AU - Elie Fadel AU - Stephen P. Hoole AU - David P. Jenkins AU - David G. Kiely AU - Nick H. Kim AU - Irene M. Lang AU - Michael M. Madani AU - Hiromi Matsubara AU - Aiko Ogawa AU - Jaquelina S. Ota-Arakaki AU - Rozenn Quarck AU - Roela Sadushi-Kolici AU - Gérald Simonneau AU - Christoph B. Wiedenroth AU - Bedrettin Yildizeli AU - Eckhard Mayer AU - Joanna Pepke-Zaba Y1 - 2021/01/01 UR - http://openres.ersjournals.com/content/early/2021/06/17/23120541.00850-2020.abstract N2 - Background Pulmonary endarterectomy (PEA), pulmonary arterial hypertension (PAH) therapy, and balloon pulmonary angioplasty (BPA) are currently accepted therapies for chronic thromboembolic pulmonary hypertension (CTEPH). This international CTEPH registry identifies clinical characteristics of patients, diagnostic algorithms, and treatment decisions in a global context.Methods 1010 newly diagnosed consecutive patients were included into the registry between February 2015 and September 2016. Diagnosis was confirmed by right heart catheterisation, ventilation-perfusion lung scan, computerised pulmonary angiography, and/or invasive pulmonary angiography after at least 3 months on anticoagulation.Results Overall, 649 patients (64.3%) were considered for PEA, 193 (19.1%) for BPA, 20 (2.0%) for both PEA and BPA and 148 (14.7%) for PAH therapy only. Reasons for PEA inoperability: technical inaccessibility (n=235), co-morbidities (n=63), and patient refusal (n=44). In Europe, America and other countries (AAO), 72% of patients were deemed suitable for PEA whereas in Japan, 70% of patients were offered BPA as first choice. Gender was evenly balanced, except in Japan where 75% of patients were female. A history of acute pulmonary embolism was reported for 65.6% of patients. At least one PAH therapy was initiated in 35.8% of patients (26.2% of PEA candidates, 54.5% of BPA candidates, and 54.1% of those not eligible for either PEA or BPA). At the time of analysis, 39 patients (3.9%) had died of PH-related causes (3.5% after PEA and 1.8% after BPA).Conclusions The registry revealed noticeable differences in patient characteristics (rates of pulmonary embolism and gender) and therapeutic approaches in Japan compared with Europe and AAO.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Guth reports personal fees from Actelion, personal fees from Bayer, personal fees from GSK, personal fees from MSD, personal fees from Pfizer, outside the submitted work;.Conflict of interest: Dr. D'Armini reports personal fees from Actelion, personal fees from Bayer, personal fees from MSD, outside the submitted work;.Conflict of interest: Dr. Delcroix reports grants and personal fees from Actelion, personal fees from Bayer, personal fees from MSD, personal fees from Reata, personal fees from Bellarophon, outside the submitted work;.Conflict of interest: Dr. Nakayama has nothing to disclose.Conflict of interest: Dr. Fadel has nothing to disclose.Conflict of interest: Dr. Hoole has nothing to disclose.Conflict of interest: Dr. Jenkins reports personal fees from Actelion, grants and personal fees from Bayer, outside the submitted work;.Conflict of interest: Dr. Kiely reports grants, personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Bayer, grants, personal fees and non-financial support from GSK, personal fees and non-financial support from MSD, outside the submitted work;.Conflict of interest: Dr. Kim reports personal fees from Actelion, personal fees from Bayer, personal fees from Merck, grants from United Therapeutics, grants from SoniVie, outside the submitted work;.Conflict of interest: Dr. Lang reports grants and personal fees from Actelion, grants and personal fees from AOPOrphan Pharma, personal fees from MSD, non-financial support from Medtronic, during the conduct of the study; grants and personal fees from Actelion, grants and personal fees from AOPOrphan, personal fees from MSD, personal fees from Ferrer, outside the submitted work.Conflict of interest: Dr. Madani reports and Consultant: ActelionConflict of interest: Dr. Matsubara reports personal fees from Actelion, personal fees from AOP orphan Pharmaceuticals AG, personal fees from Bayer, personal fees from Glaxo Smith Kline, personal fees from Pfizer Japan, Inc, personal fees from United Therapeutics, personal fees from Nippon Shinyaku, Co, Ltd, personal fees from Kaneka Medix Corporation, outside the submitted work;.Conflict of interest: Dr. Ogawa reports personal fees from Nippon Shinyaku Co., Ltd., outside the submitted work;.Conflict of interest: Dr. Ota-Arakaki has nothing to disclose.Conflict of interest: Dr. Quarck has nothing to disclose.Conflict of interest: Dr. Sadushi-Kolici has nothing to disclose.Conflict of interest: Dr. Simonneau has nothing to disclose.Conflict of interest: Dr. Wiedenroth reports personal fees from Actelion, personal fees from AOP, personal fees from Bayer, personal fees from MSD, personal fees from Pfizer, outside the submitted work;.Conflict of interest: Dr. Yildizeli has nothing to disclose.Conflict of interest: Dr. Mayer reports personal fees from Actelion, personal fees from Bayer, personal fees from MSD, personal fees from BMS, outside the submitted work;.Conflict of interest: Dr. Pepke-Zaba reports personal fees and non-financial support from Actelion, personal fees and non-financial support from MERCK, non-financial support from GSK, outside the submitted work;. ER -