What is the recommended management for bleeding risk in patients with Von Willebrand (VWD) disease undergoing surgery?

Management of Bleeding Risk in Von Willebrand Disease Patients Undergoing Surgery

For patients with Von Willebrand Disease (VWD) undergoing surgery, desmopressin (DDAVP) at 0.3 μg/kg should be administered for mild to moderate Type 1 VWD, while VWF-containing concentrates are recommended for Type 2, Type 3, and severe Type 1 VWD patients. 1, 2

Risk Assessment and Preoperative Planning

VWD Type and Severity Considerations

• Type 1 VWD (mild to moderate):

  • DDAVP is first-line therapy when factor VIII levels are >5% 1
  • Administer 30 minutes prior to scheduled procedure 1

• Type 2 VWD, Type 3 VWD, or severe Type 1 VWD:

  • VWF-containing concentrates are indicated 2
  • DDAVP is ineffective or contraindicated in these cases 1

Surgical Risk Stratification

• Minor procedures: Maintain hemostatic levels for 3-5 days 3
• Major procedures: Maintain hemostatic levels for 7-14 days 3

Treatment Protocol

DDAVP Administration (for eligible patients)

• Dosage: 0.3 μg/kg diluted in 50 ml saline and infused over 30 minutes 2
• Timing: Administer 30 minutes before surgery 1
• Monitoring: Check bleeding time, factor VIII activity, ristocetin cofactor activity, and VWF antigen during administration 1
• Limitations:
  • May repeat doses at 12-24 hour intervals 2
  • Tachyphylaxis may occur after 3-5 doses 2

VWF-Containing Concentrates

• Indications:
  • Severe VWD (Type 3)
  • Type 2 VWD
  • Type 1 VWD where DDAVP is inadequate or contraindicated 2
  • Emergency surgery requiring immediate correction 3

Laboratory Monitoring

• Parameters to monitor:
  • Factor VIII coagulant activity
  • VWF levels
  • Bleeding time 1
• Frequency: Regular monitoring throughout the perioperative period, especially for major surgeries 4

Special Considerations

Potential Complications

• Thrombotic risk:

  • Monitor for thrombotic complications, especially with repeated doses of factor concentrates 3
  • Consider thromboprophylaxis when appropriate 2

• DDAVP-related complications:

  • Fluid retention
  • Hyponatremia (especially in children) 5
  • Implement fluid restriction protocols when using DDAVP 5

Adjunctive Therapies

• Antifibrinolytic agents:
  • Consider tranexamic acid as adjunctive therapy 2
  • Particularly useful for mucosal surgeries (ENT, dental, gynecological) 6

Management Algorithm

1. Determine VWD type and severity
2. Select appropriate therapy:
   • Type 1 (mild-moderate) with FVIII >5%: DDAVP
   • Type 2, Type 3, or severe Type 1: VWF concentrates
3. Administer therapy:
   • DDAVP: 0.3 μg/kg 30 minutes before procedure
   • VWF concentrates: Dosing based on weight and severity
4. Monitor response:
   • Factor VIII levels
   • VWF activity
   • Clinical bleeding
5. Maintain therapy:
   • Minor procedures: 3-5 days
   • Major procedures: 7-14 days

Pitfalls to Avoid

• Underdosing: Inadequate replacement therapy is a common cause of surgical bleeding
• Overreliance on DDAVP in patients with severe disease or Type 2 variants
• Failure to monitor FVIII levels which may accumulate with repeated dosing of VWF concentrates 3
• Neglecting fluid management when using DDAVP, especially in pediatric patients 5

Remember that emergency surgeries require immediate correction with combined VWF and FVIII concentrates, while elective procedures allow for more tailored approaches 3.