Diagnosis of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism
- 1Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands
- 2Département de Médecine Interne et Pneumologie, Centre Hospitalo-Universitaire de Brest, Univ Brest, Brest, France
- 3Department of Respiratory Diseases, University Hospitals and Respiratory Division, Department of Chronic Diseases, Metabolism & Aging, KU Leuven – University of Leuven, Leuven, Belgium
- 4Université Paris-Saclay, Faculté de Médecine, Le Kremlin-Bicêtre, France
- 5Service de Pneumologie et Soins Intensifs Respiratoires, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre, France
- 6INSERM UMR S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
- Frederikus A. Klok, Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, the Netherlands; Albinusdreef 2, 2300RC, Leiden, the Netherlands. E-mail: f.a.klok{at}LUMC.nl
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is the most severe long-term complication of acute pulmonary embolism (PE). Untreated, CTEPH is fatal, but, if diagnosed in time, successful surgical (pulmonary endarterectomy), medical (pulmonary hypertension drugs) and/or interventional (balloon pulmonary angioplasty) therapies have been shown to improve clinical outcomes, especially in case of successful pulmonary endarterectomy. Early diagnosis has however been demonstrated to be challenging. Poor awareness for the disease by patients and physicians, high prevalence of the post-PE syndrome (i.e. persistent dyspnoea, functional limitations and/or decreased quality of life following an acute PE diagnosis), lack of clear guideline recommendations as well as inefficient application of diagnostic tests in clinical practice lead to a reported staggering diagnostic delay of longer than 1 year. Hence, there is a great need to improve current clinical practice and diagnose CTEPH earlier. In this review, we will focus on the clinical presentation of and risk factors for CTEPH, and provide best practices for PE follow-up programs from expert centres, based on a clinical case.
Footnotes
This 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: Frederikus 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.
Conflict of interest: Dr. Couturaud reports grants from Pfizer, personal fees from Bayer, other from Boeringher, personal fees and other from BMS, other from Daiichi, personal fees and other from Aztra zeneca, other from GSK, personal fees from leopharma, other from actelion, outside the submitted work.
Conflict of interest: Dr. Delcroix reports grants and personal fees from Actelion/J&J, personal fees from Bayer, personal fees from MSD, personal fees from Reata, personal fees from Bellarophon, personal fees from Acceleron, outside the submitted work.
Conflict of interest: Dr. Humbert reports personal fees from Acceleron, personal fees from Actelion, grants and personal fees from Bayer, grants and personal fees from GSK, personal fees from MSD, personal fees from United Therapeutics, outside the submitted work.
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- Received January 28, 2020.
- Accepted March 3, 2020.
- Copyright ©ERS 2020