Current strategies for managing CTEPH: results of the worldwide prospective CTEPH Registry
- Stefan Guth1⇑,
- Andrea M. D'Armini2,
- Marion Delcroix3,
- Kazuhiko Nakayama4,
- Elie Fadel5,
- Stephen P. Hoole6,
- David P. Jenkins6,
- David G. Kiely7,
- Nick H. Kim8,
- Irene M. Lang9,
- Michael M. Madani10,
- Hiromi Matsubara11,
- Aiko Ogawa11,
- Jaquelina S. Ota-Arakaki12,
- Rozenn Quarck3,
- Roela Sadushi-Kolici9,
- Gérald Simonneau13,
- Christoph B. Wiedenroth1,
- Bedrettin Yildizeli14,
- Eckhard Mayer1 and
- Joanna Pepke-Zaba6
- 1Department of Thoracic Surgery, Kerckhoff Heart and Lung Center, Bad Nauheim, Germany
- 2Cardiac Surgery, Heart-Lung Transplantation and CTEPH, University of Pavia, School of Medicine, Foundation I.R.C.C.S. Policlinico San Matteo, Pavia, Italy
- 3Clinical Department of Respiratory Diseases, University Hospitals of Leuven and Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department of Chronic Diseases and Metabolism (CHROMETA), KU Leuven – University of Leuven, Leuven, Belgium
- 4Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan
- 5Research and Innovation Unit, INSERM UMR-S 999, Marie Lannelongue Hospital, Univ Paris Sud, Paris-Saclay University, Le Plessis Robinson, France; Department of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Marie Lannelongue Hospital, Univ Paris Sud, Paris-Saclay University, Le Plessis Robinson, France; Paris-Sud University and Paris-Saclay University, School of Medicine, Kremlin-Bicêtre, France
- 6Royal Papworth Hospital, Cambridge, UK
- 7Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
- 8Division of Pulmonary and Critical Care Medicine, University of California, San Diego, La Jolla, California, USA
- 9Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Vienna, Austria
- 10Cardiovascular and Thoracic Surgery, University of California, San Diego, La Jolla, CA, USA
- 11National Hospital Organization Okayama Medical Center, Okayama, Japan
- 12Pulmonary Circulation Group and Pulmonary Function and Exercise Physiology Unit, Division of Respiratory Diseases, Department of Medicine, Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil
- 13Assistance Publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Bicêtre, Université Paris-Sud, Laboratoire d'Excellence en Recherche sur le Médicament et Innovation Thérapeutique, and Institut National de la Santé et de la Recherche Médicale Unité 999, Le Kremlin-Bicêtre, France
- 14Department of Thoracic Surgery, Marmara University School of Medicine, Istanbul, Turkey
- Stefan Guth (s.guth{at}kerckhoff-klinik.de)
Abstract
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.
Footnotes
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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: Actelion
Conflict 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;.
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- Received November 13, 2020.
- Accepted June 12, 2021.
- Copyright ©The authors 2021
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