Von Willebrand Disease (VWD) Type 3:
Classification and Inheritance
- Type 3 VWD:
- Severe quantitative deficiency of von Willebrand factor (VWF).
- Associated with the most severe bleeding phenotype among VWD types.
- Inheritance:
- Autosomal recessive inheritance.
- Genetic counselling is crucial to address risks of transmission and expression variability.
Risks in Pregnancy to Mother and Baby
- Maternal Risks:
- Significantly increased risk of antepartum, primary, and secondary postpartum haemorrhage (PPH).
- Type 3 women are at higher risk due to very low or absent VWF levels.
- Counselling:
- Essential to inform patients about the heightened bleeding risks throughout pregnancy and delivery.
Prepregnancy and Antenatal Management
- Pre-Conception:
- Comprehensive assessment of bleeding phenotype and diagnosis review.
- Multidisciplinary care approach required.
- Testing:
- VWF antigen, activity levels, and factor VIII levels checked at booking, third trimester, and before invasive procedures.
- Management:
- Referral to high-risk obstetric centres with haemophilia specialists.
- Ensure facilities for laboratory monitoring of VWF and factor VIII levels.
- Target factor VIII and VWF ristocetin cofactor (VWF:RCo) levels ≥0.5 IU/ml for invasive procedures.
- Treatment:
- DDAVP is not effective in Type 3 and should not be used.
- Use VWF replacement therapy with plasma-derived concentrates containing both VWF and factor VIII.
- Target peak VWF activity levels of 1.0 IU/ml and maintain ≥0.5 IU/ml until haemostasis is achieved.
Intrapartum Management
- Treatment Timing:
- Administer VWF replacement therapy close to delivery.
- Monitor pre- and post-treatment VWF and factor VIII levels.
- Additional Therapies:
- Tranexamic acid can be given orally or intravenously.
- Platelet transfusions may be needed in specific situations.
- Delivery Mode:
- Spontaneous vaginal delivery preferred if no other obstetric concerns.
- Avoid fetal interventions such as fetal blood sampling (FBS), ventouse, and midcavity forceps to minimise trauma for at-risk neonates.
Analgesia and Anaesthesia
- Neuraxial Anaesthesia:
- Contraindicated due to the inability to correct haemostatic defects effectively.
- Precautions:
- Discussion with senior anaesthetist required for alternative pain management.
- Avoid procedures with high bleeding risk unless VWF levels are corrected.
- Medication:
- Postnatal NSAIDs and intramuscular injections should only be used when VWF activity and factor VIII levels are >0.5 IU/ml.
Postpartum Management
- Monitoring:
- Maintain VWF and factor VIII levels >0.5 IU/ml for:
- At least 5 days following caesarean or instrumental delivery.
- At least 3 days following uncomplicated vaginal delivery.
- Medications:
- Prolonged administration of tranexamic acid may be required (e.g., 2–3 weeks or longer).
- Awareness:
- Patients should report delayed or excessive bleeding.
- Regular haemoglobin monitoring is necessary.
- Severe Cases:
- Type 3 patients may need extended VWF concentrate therapy (2–3 weeks or longer postpartum).
Neonatal Management
- Testing:
- Cord blood samples for VWF activity must be taken at birth.
- Vitamin K:
- Administer orally unless VWF activity is confirmed normal.
- Special Cases:
- Routine cranial imaging recommended for neonates with Type 3.
- Short-term prophylaxis with factor concentrate should be considered if delivery trauma is significant.
References: RCOG Green-top Guidelines No. 71 (2017).
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