Von Willebrand disease
ICD-10 D68.0 ICD-11 3B12

Management of Von Willebrand Disease in Patients Undergoing Major Surgery

Patients with Von Willebrand disease (VWD) who require major surgery face a distinct perioperative challenge: ensuring adequate haemostatic coverage across the surgical and postoperative period. Standard factor replacement strategies used in other bleeding disorders may be insufficient without attention to the full coagulation factor profile.

Clinical Scenario

This protocol addresses VWD patients scheduled for major surgery — a situation where sustained haemostatic control in the perioperative window is critical. Both the type of bleeding disorder and the magnitude of the surgical procedure inform the monitoring and replacement targets required.

Treatment Goals

The primary targets are maintaining both coagulation factor activity levels — not a single factor in isolation — at adequate thresholds for a minimum duration after surgery. Meeting both targets simultaneously is the defining requirement of this protocol.

Treatment Approach (Partial Overview)

Management involves VWF concentrate to support both relevant factor activity levels through the postoperative period. Relying on a single factor target is explicitly not recommended.

Full regimen details — including targets, duration, monitoring, and decision points — are available in the structured protocol.

References

  • In patients with VWD undergoing major surgery, should the FVIII level be kept at ≥0.50 IU/mL for at least 3 days after surgery, or should the VWF activity level be kept at ≥0.50 IU/mL for at least 3 days after surgery?
  • The panel suggests targeting both FVIII and VWF activity levels of ≥0.50 IU/mL for at least 3 days after surgery (conditional recommendation based on very low certainty in the evidence of effects).
  • The panel suggests against using only FVIII ≥0.50 IU/mL as a target level for at least 3 days after surgery (conditional recommendation based on very low certainty in the evidence of effects).
DOI: 10.1182/bloodadvances.2020003264. View source ↗