Perioperative Management of Von Willebrand Disease (VWD)

Introduction

• Surgical procedures present a hemostatic challenge for VWD patients.
• Perioperative management is crucial to reducing bleeding risk.
• Key factors include surgery type, baseline von Willebrand factor (VWF) levels, bleeding history, and previous challenge responses.

Risk Stratification and Management

• Baseline bleeding scores (BSs) help identify high-risk patients.
• Lack of consensus on therapeutic targets and assays for monitoring post-surgery.
• Hemostatic levels maintained until bleeding risk reduces.

Hemostatic Supplementation

• More complex in VWD due to combined VWF and factor VIII (FVIII) deficiency.
• Emergency surgery requires co-administration of VWF and FVIII.
• Elective procedures benefit from early VWF infusion.

Clinical Case

• 70-year-old male with type 2M VWD requiring knee replacement.
• Historical records show significant bleeding history.
• DDAVP and tranexamic acid (TXA) previously used.

Characterization of VWD

• VWD includes quantitative (type 1 and 3) and qualitative (type 2) deficiencies.
• Bleeding diathesis includes mucocutaneous bleeding.
• Phenotypic variability exists even within subtypes.
• Use of bleeding assessment tools (BATs) for evaluation.

Preoperative Assessment

• Plasma VWF levels can increase with age.
• Repeat testing necessary to guide hemostatic plans.
• Significant reduction in VWF levels in the case study.

Stratification of Surgical Risk

• Defining treatment plans for elective procedures is essential.
• Higher target VWF levels required for major surgeries.

Treatment Options

• Achievable through DDAVP or exogenous VWF infusion.
• Antifibrinolytic agents like TXA are useful.

Antifibrinolytic Therapy

• Reduces bleeding risk by stabilizing clots.
• Clinical trials show no significant increase in thromboembolism risk.
• Recommended for surgeries unless contraindicated.

Role of DDAVP

• Stimulates VWF release; effective in low VWF levels.
• Not suitable for type 2B VWD due to thrombocytopenia concerns.
• Used for mild defects and short hemostatic cover.

pdVWF Concentrates

• Differ in production and FVIII content.
• Repeated doses needed for major surgeries.
• Can lead to elevated FVIII:C levels post-surgery.

Recombinant VWF (rVWF)

• Recent licensure for surgical prophylaxis.
• Contains increased ultralarge multimers.

Hemostatic Management Plan

• Major surgeries require specialist centers.
• Written plans communicated to patient and team.

Perioperative Management

• TXA and pdVWF-FVIII concentrates recommended.
• rVWF requires preoperative administration.

Perioperative Therapeutic Targets

• Aim to maintain hemostatic VWF levels post-surgery.
• Varied guidelines on optimal plasma levels.

Thromboprophylaxis

• Consider thrombosis risk when using VWF replacement.
• Current guidelines suggest avoiding high FVIII:C levels.

Pharmacokinetic Guided Dosing

• Potential for personalized treatment but requires further research.

Postoperative Bleeding

• Consider surgical causes if VWF and FVIII:C levels are adequate.
• Platelet transfusion may aid hemostasis.

Case Outcome

• Successful management with TXA and VWF replacement.
• No bleeding complications post-surgery.

Conclusion

• Complex perioperative management requiring personalized approaches and specialist guidance.
• Monitoring and guided dosing ensure adequate plasma levels and risk management.