Abstract
Plasma samples from 8 children with ventricular septal defect (VSD) were previously found to lack the high molecular weight vWf multimers by electrophoresis in SDS-agarose followed by staining with 125I-radiolabeled anti-vWf and autoradiography. Low vWf by antigenic and ristocetin cofactor assays were found in 4 whereas all had normal VIII:C; none had evidence of intravascular coagulation. To determine if these children were different from others with VSD, cardiac catheterization findings were evaluated. All had a sealed foramen ovale and high or high normal end diastolic pulmonary artery pressure. To date, 3 of the children have been re-studied after surgical correction of their VSD. Two had normalization of vWf multimers; the third, whose vWf was abnormal postoperatively, had a residual pressure gradient across a previous pulmonary artery banding site. Multimeric abnormalities were not found in the parents of 3 patients. Thus, some patients with VSD and an abnormal hemodynamic state may have an “acquired” loss of the high vWf multimers that is normalized with correction of the hemodynamic defect.
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