A case of acquired von Willebrand disease due to pulmonary stenosis after Jatene's operation

The case of a 9-year-old girl with pulmonary stenosis complicated by acquired bleeding tendency after Jatene's operation is reported. Coagulation study revealed that platelet count and von Willebrand factor were reduced. Catheterization study revealed severe pulmonary stenosis, the pressure gradient between the right ventricle (RV) and pulmonary artery (PA) being 190 mmHg. A link between pulmonary stenosis and bleeding tendency was suggested and the patient was diagnosed as having acquired von Willebrand disease due to activated platelet/von Willebrand factor interactions enhanced by "shear stress" at the site of pulmonary stenosis. Right ventricular outflow reconstruction was done when she was 9 years-old. Due to a protocol based on the results of the challenge test that we instituted to determine the efficacy and effective duration of blood derivatives and hemostatic agents, perioperative massive bleeding was avoided. Bleeding tendency disappeared and coagulation study findings normalized with correction of the abnormal hemodynamic state.