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RESEARCH PRODUCT

Determinants of diagnostic delay in chronic thromboembolic pulmonary hypertension: results from the European CTEPH Registry.

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Chronic thromboembolic pulmonary hypertension (CTEPH) is characterised by chronic thrombi in the pulmonary arterial bed, causing pulmonary hypertension [1–3]. CTEPH is diagnosed in ∼3% of patients who survive a symptomatic acute pulmonary embolism (PE) [4]. While the surgical removal of chronic fibrotic thrombotic vascular occlusions by pulmonary endarterectomy (PEA) may cure most patients with CTEPH by normalising pulmonary artery hemodynamics and improving symptoms, patients who remain not operated or do not undergo balloon pulmonary angioplasty have severe functional limitations, and poor quality of life and survival [5, 6]. Since the natural course of CTEPH involves progressive remodelling of the distal arteries and increase of pulmonary vascular resistance (PVR), which are both important determinants of outcome, early CTEPH diagnosis and referral to an expert centre are both critical for optimal treatment. It has however been shown that early diagnosis of CTEPH remains a major challenge, as demonstrated by a median diagnostic delay of 14 months identified in the large European CTEPH registry [7]. The most likely explanations for this are diagnostic misclassifications as acute PE or other conditions, the nonspecific and often insidious clinical presentation of CTEPH, and the cumbersome diagnostic process of CTEPH, which involves multiple healthcare providers from different clinical specialties [8, 9]. The main determinants for and consequences of the diagnostic delay in CTEPH have not been studied. 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: Dr. Barco reports non-financial support from Bayer HealthCare and Daiichi Sankyo, outside the submitted work. Conflict of interest: Dr. Dartevelle has nothing to disclose. Conflict of interest: Marion Delcroix has been investigator, speaker, consultant, or steering committee member for Actelion, Bayer, Eli Lilly, GlaxoSmithKline, MSD, and Pfizer; and has received research grants from Actelion. Conflict of interest: Dr. Fadel has nothing to disclose. Conflict of interest: Dr. Jenkins reports grants and personal fees from Bayer, personal fees from Actelion, outside the submitted work. Conflict of interest: Nick Kim reports research support form Actelion, Bayer, J&J and Merck, and received lecture honoraria from Bayer Conflict of interest: Frederikus Klok reports research grants from Bayer, Bristol-Myers Squibb, Boehringer-Ingelheim, MSD, Daiichi-Sankyo, Actelion, the Dutch thrombosis association and the Dutch Heart foundation Conflict of interest: Stavros Konstantinides has received consultancy and lecture honoraria from Bayer HealthCare, Boehringer Ingelheim, Daiichi-Sankyo, and Pfizer -Bristol-Myers Squibb; payment for travel accommodation/meeting expenses from Bayer HealthCare; and institutional grants from Boehringer Ingelheim, Bayer HealthCare, and Daiichi-Sankyo. Conflict of interest: Dr. Lang reports personal fees from MSD, grants and personal fees from AOPORphan Pharma, grants and personal fees from Actelion, outside the submitted work. Conflict of interest: Dr. Madan has nothing to disclose. Conflict of interest: Dr. Matsubara reports personal fees from Actelion Pharmaceuticals Japan, Ltd., personal fees from AOP Orphan Pharmaceuticals AG, personal fees from Bayer Yakuhin, Ltd., personal fees from Nippon Shinyaku, Co., Ltd., personal fees from Pfizer Japan, Inc., personal fees from Kaneka Medix Corporation, outside the submitted work. Conflict of interest: Dr. Mayer reports personal fees from Actelion, personal fees from Bayer, personal fees from MSD, personal fees from Pfizer, outside the submitted work. Conflict of interest: JPZ or her institution have received research, educational grants from Actelion, Merck, Bayer. JPZ served at advisory boards Actelion, J&J, Merck, Bayer, GSK