Open-heart surgery in von Willebrand's disease

The case is presented of a hemophilia carrier, also affected by von Willebrand's disease, who underwent aortic valve replacement. The clinical and laboratory findings of von Willebrand's disease (prolonged bleeding time, low factor VIII, and abnormal platelet activity) are discussed, and a protocol for management of patients with low factor VIII levels (such as hemophilia carriers and subjects with hemophilia A or von Willebrand's disease) undergoing open-heart surgery is proposed. Our case proves that corrective cardiac surgery in similar circumstances is feasible so long as adequate levels of factor VIII are maintained.