Continuous Infusion of Von Willebrand Factor and Factor Viii After Elective Heart Surgery in a 12-Year-Old Girl With Von Willebrand Disease Type 3

Abstract

The continuous infusion of von Willebrand factor (VWF) in a 12-year-old girl with type 3 von Willebrand disease is described. The patient had elective heart surgery with cardiopulmonary bypass for closure of her atrial septum defect. The surgical procedure lasted 3 h. A presurgical bolus followed by a postoperative continuous infusion of factor VIII/VWF concentrates was administered. During the continuous infusion of clotting factors, stable plasma levels of hemostasis and avoidance of dangerously low levels of factor concentrates were achieved. No peri- or postsurgical complications occurred. Continuous infusion of clotting factors allows constant and hemostatic factor concentrations to be maintained with the possibility of dose titration and adjustment.

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

“…For all eight patients, treatment with continuous infusion consumed 50% less factor concentrate compared with bolus dosing, and no bleeding or thrombotic events were observed. Kuhne et al reported the successful use of continuous infusion of VWF/FVIII concentrate (Haemate ® HS/Humate-P ® ) in a 12-year-old patient with type 3 VWD undergoing elective cardiopulmonary bypass surgery; no evidence of infection, bleeding or thromboembolic complications was observed [25]. 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].…”

“…Although numerous studies have described the efficacy of continuous infusion in haemophilia A and B patients (reviewed in ), the efficacy of continuous infusion in VWD has been limited to case studies . Because of concern for the potential development of inhibitors while treating patients with coagulation disorders, and limited data on the use of continuous infusion in VWD patients, the current prospective study was undertaken to investigate inhibitor development with continuous infusion of factor concentrates during and after surgery in subjects with VWD, haemophilia A or haemophilia B. Observations were made on the hemostatic efficacy, pharmacokinetic profiles, inhibitor development, safety, virus seroconversions and product usage.…”

Haemate®P von Willebrand factor/factor VIII concentrate: 25 years of clinical experience

“…Haemate P has also been reported to be effective in acquired VWD [66]. The concentrate has been shown to be effective not only when administered as a bolus dose, as is customary, but also as a continuous infusion [60,64,[66][67][68][69][70][71][72]. Starting in the 1990s, efforts to delineate the pharmacokinetic properties of Haemate P in VWD patients were undertaken in order to optimize the dose size and frequency of administration [47,62,[73][74][75][76].…”

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

“…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].…”