Closure of a ventricular septal defect in a patient with von Willebrand disease

Few reports of open heart surgery being performed in patients with von Willebrand disease (vWD) have been documented. We describe herein the case of a 5-year-old girl with a ventricular septal defect (VSD) and vWD who underwent patch closure under cardiopulmonary bypass (CPB). The hematological parameters relating to vWD, namely, factor VIII, von Willebrand factor, and factor VIII-related antigen were monitored perioperatively. These laboratory findings were elevated after the termination of CPB, and consistently maintained within the normal range for 4 months postoperatively. The operation was successfully completed without the administration of factor VIII concentrates or homologous blood products.