Perioperative Management of Type 1 von Willebrand Disease

First‑Line Therapy – Desmopressin (DDAVP)

The American Society of Anesthesiologists recommends desmopressin 0.3 µg/kg as the initial hemostatic agent for patients with type 1 VWD undergoing surgery; VWF/FVIII concentrates are reserved for non‑responders or when DDAVP is insufficient【1】【2】【3】.
Desmopressin should be diluted in 50 mL of saline and infused over 30 minutes to achieve optimal plasma levels【4】【2】【3】.
The drug works by stimulating endothelial release of endogenous VWF, which then binds platelets to collagen via GP1b receptors and to other platelets via GPIIb/IIIa receptors【2】【3】.
For minor surgical procedures, DDAVP alone or combined with tranexamic acid provides adequate hemostasis in the majority of type 1 VWD patients【5】.
In cardiac surgery, pre‑operative administration of DDAVP improves peri‑operative hemostasis in VWD patients【6】【7】.

When the desmopressin response is inadequate or contraindicated, specific VWF/FVIII concentrates should be used as the next line of therapy【1】【7】.
ASA guidelines state that bleeding patients with type 1 VWD should receive desmopressin first, followed by VWF/FVIII concentrate if there is no adequate response or if desmopressin is unavailable【1】.
Target plasma levels of VWF and FVIII should be maintained at ≥ 50 IU/dL for neuraxial anesthesia and major surgical procedures【8】.

Tranexamic acid should be added to desmopressin or VWF/FVIII concentrate to enhance hemostatic efficacy in type 1 VWD patients undergoing surgery【5】.
Small series report hemostatic efficacy > 90 % when tranexamic acid is combined with DDAVP【5】.

If VWF/FVIII concentrate cannot be obtained and desmopressin fails, cryoprecipitate (which contains VWF and fibrinogen) is an acceptable alternative【1】.

Prophylactic DDAVP should not be given to patients without a documented VWD or platelet hemostatic defect; meta‑analyses show no benefit in cardiac surgery patients without bleeding disorders【6】【7】.