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

Abstract

SummaryWe 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 ( 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 intraoperative 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. The management of adult patients with von Willebrand's disease has only marginally been described in the available literature since 1983, especially in the context of coronary artery bypass graft. This reflects the small number of patients with an established diagnosis of von Willebrand's disease and the even smaller number of these presenting for open heart surgery. We describe the peri-operative management of a patient with type 2N von Willebrand's disease presenting for coronary artery bypass graft. A literature search revealed a few studies reporting the association of aortic stenosis and acquired von Willebrand's disease [1-3], but only one report in the anaesthetic literature [4], and two reports in the non-anaesthetic literature describing heart surgery in children [5,6]. Only a few details about management of this bleeding disorder have been offered [4,7,8]. Case reportA 75-year-old male patient was scheduled for elective coronary artery bypass graft for severe three-vessel coronary artery disease with involvement of the left main stem. The patient was known for many years to have type 2N von Willebrand's disease and was under the care of the haematology service of the University Medical Center, Utrecht.On the early morning of the day of surgery his factor VIII baseline activity was 53%, von Willebrand factor antigen 29% and von Willebrand factor ristocetin 16%. According to the peri-operative protocols of our haematology service a pre-operative bolus of 3000 IU factor VIII ⁄ Willebrand factor concentrate (Haemate-P ) was then commenced and the patient proceeded to coronary surgery with full heparinisation and cardiopulmonary bypass. Intra-operatively, no bleeding complications occurred and administration of blood products was not deemed necessary. Postoperatively the patient was transferred to the intensive care unit (ICU) for further care with the

No inhibitor development after continuous infusion of factor concentrates in subjects with bleeding disorders undergoing surgery: a prospective study

“…In another case study, five patients with type 3 VWD achieved 'good' hemostatic control following continuous infusion of high-purity FVIII/VWF concentrate (BPL-8Y) for surgery, trauma or epistaxis [32]. Finally, continuous infusion of VWF/FVIII concentrate (Haemate ® HS/Humate-P ® ) was also efficacious for a patient with Type 2N VWD who underwent coronary artery bypass graft surgery, and despite the patient's FVIII and VWF levels exceeding recommended values, no complications were observed [33].…”

Hepatectomy in patients with inherited blood coagulation disorders can be safely performed with adequate coagulation factor replacement

“…The reason for measuring the pharmacokinetics is to calculate the amount of recombinant FVIII concentrate required for each patient, which allows precise management during surgery and may help to prevent both postoperative haemorrhage and thromboembolism. The reported number of patients with VWD is even smaller than that of patients with haemophilia . The present study is one of the largest case series of hepatectomy in patients with concurrent HCC and haemophilia or VWD undergoing standardized perioperative management.…”

“…Surgery can be safely performed using VWF/factor VIII (FVIII) concentrate. However, little is known regarding liver resection for which the bleeding risk is considered to be one of the highest among all major surgeries, in patients with haemophilia and VWD …”

Redo-redo aortic root replacement with a mechanical valved conduit in a patient with von Willebrand's disease: Case report

“…The replacement therapy can be monitored by factor assays performed in a specialist haemostasis laboratory [5]. Such assays require a turnaround time of approximately one hour and are essential to enable optimal control of factor levels and dosing.…”