2025 Guidelines for the Evaluation and Management of Adults With Congenital Heart Disease
December 19, 2025
Brand Name :
Vonvendi
Synonyms :
von Willebrand factor, recombinant
Class :
Coagulation Factors; Hemostatics
Dosage Forms & Strengths
Lyophilized powder for reconstitution
650 IU
1300 IU
Safety and efficacy are not seen in pediatrics
Refer to the adult dosing
Actions and Spectrum:
The recombinant Von Willebrand factor (VWF) is a medication used to treat bleeding episodes in patients with von Willebrand disease (VWD).
VWF, recombinant, is a synthetic version of the VWF protein produced through genetic engineering. It works by replacing the missing or defective VWF in patients with VWD, enhancing their ability to form blood clots and control bleeding.
VWF recombinant is effective in treating both types of VWD: type 1, which is a partial deficiency of VWF, and type 2, which is a qualitative defect of VWF. It is also used to treat some cases of type 3 VWD, a complete deficiency of VWF.
VWF, recombinant, is administered as an intravenous infusion, which means it is delivered directly into the bloodstream through a vein.
Frequency defined
<1%
Nausea
Tachycardia
Chest discomfort
Hot flush
Generalized pruritis
Tremor
Increased heart rate
Dysgeusia
Dizziness
Hot flush
Infusion site paresthesia
Contraindication/Caution:
Contraindications:
Cautions:
Pregnancy consideration:
No data is available regarding the use of the drug during pregnancy.
Breastfeeding warnings:
No data is available regarding the secretion of the drug in breast milk.
Pregnancy category:
Pharmacology:
Von Willebrand factor (VWF) recombinant is a synthetic version of the VWF protein produced through genetic engineering. It mimics the natural VWF protein, which is crucial in blood clotting.
VWF recombinant works by binding to and stabilizing platelets. This enhances the ability of the platelets to aggregate and adhere to damaged blood vessels, promoting the formation of a stable blood clot. VWF, recombinant, also carries and protects clotting factor VIII, another vital protein for blood clotting.
VWF, recombinant, is administered as an intravenous infusion, which means it is delivered directly into the bloodstream through a vein. It has a rapid onset of action, with peak effects seen within 30 minutes of administration. The duration of action is variable and depends on the individual patient’s response to the medication.
VWF, recombinant, is primarily used to treat bleeding episodes in patients with von Willebrand disease (VWD). It effectively treats both types of VWD: type 1, which is a partial deficiency of VWF, and type 2, which is a qualitative defect of VWF. It is also used to treat some cases of type 3 VWD, a complete deficiency of VWF.
Pharmacodynamics:
The pharmacodynamics of von Willebrand factor (VWF) recombinant involves interacting with various components of the blood clotting cascade, forming stable blood clots.
VWF, recombinant binds to platelets through its A1 domain, which promotes platelet aggregation and adhesion to the site of vascular injury. This allows the platelets to form a plug and prevent further bleeding. VWF, recombinant, also binds to and protects clotting factor VIII, which is essential for forming fibrin, a protein that stabilizes the blood clot.
The pharmacodynamics of VWF recombinant is dose-dependent, with higher doses leading to more rapid and sustained hemostatic effects. The duration of action is variable and depends on the individual patient’s response to the medication.
Pharmacokinetics:
Absorption
The area under the curve (for 0-inf) is 1541.4-2939 hr·U/dL
Elimination and Excretion
The rate of clearance is 0.03-0.04 dL/kg/hr
The half-life is 49.3-22.6 hours
Administration:
Von Willebrand factor (VWF) recombinant is administered as an intravenous infusion, delivering it directly into the bloodstream through a vein.
The dosage of VWF, recombinant, is based on the patient’s weight, severity of bleeding, and response to the medication. The medication may be given as a single dose or a series of doses over time, depending on the severity and duration of the bleeding episode.
Before administering VWF recombinant, the healthcare provider will assess the patient’s medical history, including any allergies or previous reactions to the medication. The medication should not be used in patients with known hypersensitivity or allergy to VWF, recombinant, or its components.
The infusion rate of VWF, recombinant, should be based on the patient’s tolerance and response to the medication and should not exceed the recommended rate. The healthcare provider should monitor the patient during and after the infusion for any adverse reactions, including allergic reactions, thrombotic events, or other side effects.
Patient information leaflet
Generic Name: Von Willebrand factor (recombinant)
Pronounced: von-WIL-e-brand-FAK-tor
Why do we use Von Willebrand factor (recombinant)
The recombinant Von Willebrand factor (VWF) is used to treat bleeding episodes in patients with von Willebrand disease (VWD), a genetic bleeding disorder caused by a deficiency or dysfunction of VWF.
VWF plays a crucial role in blood clotting by binding to and stabilizing platelets, which are small blood cells that help to form blood clots—in patients with VWD, the deficient or dysfunctional VWF results in impaired platelet function and reduced ability to form stable blood clots, leading to increased bleeding and bruising.
VWF recombinant works by replacing the missing or defective VWF in patients with VWD, enhancing the ability of the platelets to aggregate and adhere to damaged blood vessels, and promoting the formation of a stable blood clot. VWF, recombinant, also carries and protects clotting factor VIII, another protein that is important for blood clotting.