The ADAMTS13-VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is an important consequence of pulmonary embolism that is associated with abnormalities in haemostasis. We investigated the ADAMTS13-von Willebrand factor (VWF) axis in CTEPH, including its relationship with disease severity, inflammation,

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Conflict of interest: M. Newnham reports education support (travel, registration and accommodation) to attend conferences from MSD and GSK, outside the submitted work. Conflict of interest: K. South has nothing to disclose. Conflict of interest: M. Bleda has nothing to disclose. Conflict of interest: W.R. Auger reports grants and non-financial support for advisory board work (travel support only) from Bayer, outside the submitted work. Conflict of interest: J.A. Barberà has nothing to disclose. Conflict of interest: H. Bogaard has nothing to disclose. Conflict of interest: K. Bunclark has nothing to disclose. Conflict of interest: J.E. Cannon reports grants for educational meetings from Actelion, GSK and MSD, outside the submitted work. Conflict of interest: M. Delcroix has nothing to disclose. Conflict of interest: C. Hadinnapola has nothing to disclose. Conflict of interest: L.S. Howard reports grants from Bayer PLC, during the conduct of the study. Conflict of interest: D. Jenkins reports grants and personal fees from Bayer, and personal fees from Actelion, outside the submitted work. Conflict of interest: E. Mayer reports speaker and consultancy fees from Actelion, Bayer and MSD, and speaker fees from Pfizer, outside the submitted work. Conflict of interest: C. Ng has nothing to disclose. Conflict of interest: C.J. Rhodes has nothing to disclose. Conflict of interest: N. Screaton has nothing to disclose. Conflict of interest: K. Sheares reports educational support (travel, registration and accommodation) to attend conferences from Actelion, Bayer, MSD and GSK, and has been on an advisory board for Actelion, outside the submitted work. Conflict of interest: M.A. Simpson has a contract of service with Genomics PLC, outside the submitted work. Conflict of interest: M. Southwood has nothing to disclose. Conflict of interest: L. Su has nothing to disclose. Conflict of interest: D. Taboada reports speaker honoraria and education/travel grants from Actelion, Bayer, GlaxoSmithKline, Lilly, MDS and Pfizer, outside the submitted work. Conflict of interest: M. Traylor has nothing to disclose. Conflict of interest: R.C. Trembath has nothing to disclose. Conflict of interest: S.S. Villar has nothing to disclose. Conflict of interest: M.R. Wilkins has nothing to disclose. Conflict of interest: J. Wharton reports personal fees for advisory board work from Actelion Pharmaceuticals Ltd, outside the submitted work. Conflict of interest: S. Gräf has nothing to disclose. Conflict of interest: J. Pepke-Zaba (or her institution) has received research/educational grants and has been serving on advisory boards for Actelion, Bayer, Merck and GSK. Conflict of interest: M. Laffan reports grants from British Heart Foundation during the conduct of the study; and support to attend conferences from Shire, outside the submitted work. Conflict of interest: D.A. Lane has nothing to disclose. Conflict of interest: N.W. Morrell has nothing to disclose. Conflict of interest: M. Toshner reports grants and personal fees from Bayer, Merck and Actelion, personal fees from GSK, and grants from Roche, during the conduct of the study.