Figure 1.

von Willebrand Factor Metabolism Is Abnormal in Patients With a Glenn Circulation

(A) In an enzyme-linked immunosorbent assay (ELISA), a disintegrin and metalloproteinase thrombospondin (motif) #13 (ADAMTS-13) was significantly higher in children with a Glenn circulation than controls. (B) In an ELISA, plasma von Willebrand factor (vWF) antigen was modestly lower in children with Glenn circulation than controls. (C) Representative gel electrophoresis for vWF showed a different pattern of high-molecular-weight (HMW) vWF multimers and vWF fragments in children with a Glenn circulation than controls. The top blot (green box) showed modestly decreased HMW vWF multimers in Glenn patients versus controls. The bottom blot (blue box) showed increases in multiple vWF fragment bands, especially bands at approximately 460, 365, and 310 kDa. (D,E) HMW vWF length and density were significantly lower in children with a Glenn circulation than controls. (F) The density of vWF degradation fragments trended toward an increase in children with a Glenn circulation compared to controls. ANOVA = analysis of variance; IVC = inferior vena cava; PA = pulmonary artery.