Chronic thromboembolic pulmonary hypertension: realising the potential of multimodal management.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. Important advances have enabled better understanding, characterisation, and treatment of this condition. Guidelines recommending systematic follow-up after acute pulmonary embolism, and the insight that CTEPH can mimic acute pulmonary embolism on initial presentation, have led to the definition of CTEPH imaging characteristics, the introduction of artificial intelligence diagnosis pathways, and thus the prospect of easier and earlier CTEPH diagnosis. In this Series paper, we show how the understanding of CTEPH as a sequela of inflammatory thrombosis has driven successful multidisciplinary management that integrates surgical, interventional, and medical treatments. We provide imaging examples of classical major vessel targets, describe microvascular targets, define available tools, and depict an algorithm facilitating the initial treatment strategy in people with newly diagnosed CTEPH based on a multidisciplinary team discussion at a CTEPH centre. Further work is needed to optimise the use and combination of multimodal therapeutic options in CTEPH to improve long-term outcomes for patients.
Competing Interests: Declaration of interests MD reports research grants from Janssen; and speaker and consultancy fees from Altavant, Acceleron, AOP, Bayer, Ferrer, Gossamer, INARI, Janssen, United Therapeutics, and MSD, outside of the submitted work and all paid to her institution. She is holder of a Janssen Chair for Pulmonary Hypertension at the KU Leuven. MdP reports speaker fees and consultancy honoraria from AstraZeneca and BMS; speaker fees from Bayer; and consultancy honoraria from Merck and Roche. XJ reports research grants from Janssen, MSD, and Bayer HealthCare; and speaker fees from Janssen and MSD, outside of the submitted work. DPJ reports speaker and consultancy fees from Janssen, outside of the submitted work. IML has relationships with drug companies, including AOP-Health, Actelion-Janssen, MSD, United Therapeutics, Pulnovo, Medtronic, Neutrolis and Sanofi; in addition to being an investigator in trials involving these companies, relationships include consultancy service, research grants, and membership of scientific advisory boards. HM reports research grants from Nippon Shinyaku; speaker and consultancy fees from Bayer, Janssen, and MSD; and speaker fees from Kaneka Medix, Mochida, Nippon Shinyaku, and Niprol, outside of the submitted work. LJM participated in an advisory board on CTEPH imaging for Janssen. GS has received advisory board and speaker fees from Acceleron, Bayer, Janssen, MSD, and Merck. CBW reports speaker fees from Actelion/Janssen, AOP Orphan Pharmaceuticals AG, Bayer AG, BTG, MSD, OrphaCare, and Pfizer; and consultancy honoraria from Actelion/Janssen and MSD. NHK has received speaker fees from Bayer and Janssen; consultancy fees from Bayer, Janssen, Merck, Polarean, Pulnovo, and United Therapeutics; and research support from Altavant and Gossamer Bio, outside of the submitted work. RQ declares no competing interests.
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