Peri-operative management of an adult patient with type 2N von Willebrand's disease scheduled for coronary artery bypass graft

We describe a patient with type 2N von Willebrand's disease scheduled for elective coronary artery bypass graft for severe three-vessel coronary artery disease with involvement of the left main stem. He was given a pre-operative bolus of 3000 IU factor VIII/Willebrand factor concentrate (similar to 40 IU.kg(-1)), followed by a continuous infusion of 3 IU.h(-1) (228 IU.h(-1)) before undergoing coronary surgery with full heparinisation and cardiopulmonary bypass. There were no intra-operative bleeding complications and only one unit of packed red blood cells was required postoperatively. Thromboprophylaxis with low-molecular weight heparin and aspirin was given and the infusion of factor VIII/von Willebrand factor concentrate continued for 2 days. As a result of haematological monitoring, heparin therapy was changed from prophylactic to therapeutic on day 5-6 and stopped on day 7.