ADAMTS13 activity, D-dimer and von Willebrand factor (VWF) multimeric size in chronic thromboembolic pulmonary hypertension (CTEPH) and healthy controls. a) Specific ADAMTS13 activity (activity:antigen (Act:Ag) ratio). b) D-dimer antigen levels. c) Specific ADAMTS13 activity and D-dimer antigen correlation. Healthy control correlation: rho=0.0938, p=0.761; CTEPH correlation: rho= −0.220, p=0.313. d) VWF multimeric size (VWF collagen binding assay (CBA):Ag ratio). A subset of CTEPH patients (n=23) with the lowest ADAMTS13 antigen levels (below the first quartile of the CTEPH group) and healthy controls (n=14) were used for a–c. VWF multimeric size was measured in CTEPH (n=21) samples with the highest VWF antigen concentrations (above the third quartile of the CTEPH group) using the same healthy control subset and displayed in d. The Mann–Whitney U test was used to calculate group differences (a, b, d) and correlation was assessed using Spearman's rank correlation coefficients (c).