Management of von Willebrand Disease in Pregnancy
For pregnant women with von Willebrand disease (VWD), management should include regular monitoring of factor VIII and von Willebrand factor levels during pregnancy with targeted treatment to ensure levels above 0.50 IU/mL before delivery to minimize bleeding complications. 1
Physiological Changes in Pregnancy
- Pregnancy typically causes a progressive increase in von Willebrand factor (VWF) and factor VIII levels, which may normalize in mild cases of VWD by the third trimester 1
- Despite this increase, women with more severe forms of VWD (types 2 and 3) often maintain abnormal levels throughout pregnancy and require specific management 1
- The physiologic increase in VWF levels during pregnancy may be insufficient to prevent bleeding complications, particularly during delivery and postpartum 2
Monitoring During Pregnancy
- Factor VIII and VWF levels should be measured at baseline (early pregnancy), 28 weeks, and 34-36 weeks gestation 1
- Additional monitoring may be required before any invasive procedures 2
- Target levels for safe delivery: VWF ristocetin cofactor activity and factor VIII levels should be maintained above 0.50 IU/mL (50 IU/dL) 2
Treatment Options Based on VWD Type
Type 1 VWD (most common)
- First-line treatment: Desmopressin (DDAVP) for patients with documented response 1, 3
- Typical dosing: 0.3 mcg/kg IV approximately 30 minutes before invasive procedures or delivery 4
- DDAVP can be administered in the 36th/37th week of gestation to prepare for delivery 3
- Monitor for potential side effects including hyponatremia and fluid retention 4
Type 2 VWD
- Type 2A, 2M, 2N: Consider DDAVP if previously responsive 1
- Type 2B: DDAVP is relatively contraindicated due to risk of thrombocytopenia; VWF concentrate is preferred 1
- VWF-containing factor concentrates are generally preferred for all type 2 subtypes 1, 2
Type 3 VWD
- VWF-containing factor concentrates are the treatment of choice 1
- Higher doses may be required compared to other types 2
Labor and Delivery Management
- For vaginal delivery or cesarean section, VWF and factor VIII levels should be above 0.50 IU/mL 2
- For women receiving therapeutic-dose treatment, consider scheduled delivery with prior discontinuation of treatment 5
- Neuraxial anesthesia can be safely performed with appropriate factor correction 6
- Treatment with desmopressin or VWF/factor VIII concentrate before neuraxial anesthesia may be required (28.7% of cases in a large series) 6
Postpartum Management
- Continue monitoring and treatment for at least 2 weeks postpartum due to risk of delayed hemorrhage 2
- Tranexamic acid may be considered as adjunctive therapy for postpartum hemorrhage, though evidence quality is very low 7
- For breastfeeding women requiring anticoagulation, options include unfractionated heparin, LMWH, warfarin, acenocoumarol, fondaparinux, or danaparoid 5
Multidisciplinary Approach
- Management should involve hematology, obstetrics, and anesthesiology 6
- Pre-delivery planning should include specific protocols for factor replacement and hemostatic monitoring 6
- Postpartum care should include close follow-up for at least 6 weeks with appropriate prophylaxis based on factor levels 8
Common Pitfalls to Avoid
- Failure to monitor factor levels throughout pregnancy, especially before delivery 2
- Discontinuing treatment too soon after delivery (continue for at least 2 weeks) 2
- Inadequate factor replacement before neuraxial anesthesia 6
- Not recognizing that delayed postpartum hemorrhage may occur despite adequate prophylaxis 2