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Anesthetic Management of Von Willebrand Disease in Pregnancy
Abstract Number: T2I-328
Abstract Type: Original Research
Von Willebrand disease (vWD) is the most common heritable bleeding diathesis and involves quantitative (type 1,3) or qualitative (type 2) defects in von Willebrand factor with associated abnormalities in factor VIII and platelets. There are limited reports and no definitive guidelines on the anesthetic management of vWD in pregnancy. Here we describe the management of patients with vWD in pregnancy at a single center over a 20-year time period.
After obtaining IRB approval, we searched our institution’s Research Patient Data Registry for patients with the concurrent diagnoses of pregnancy and vWD between January 1, 2000 and January 1, 2019. Descriptive statistics were performed.
A total of 48 individual patients and 68 deliveries were identified. Mean age at the time of delivery was 30.5 years. Type of vWD and obstetric anesthesia management is as shown in the table. Neuraxial techniques were used in 58/68 deliveries. 28 deliveries (29%) were by cesarean (14 spinal, 12 epidural, 2 general anesthesia). 40 deliveries were vaginal (32 epidural). Of the patients who did not receive neuraxial anesthesia, the reasons were as follows: patient preference (4), precipitous delivery (4), and general anesthesia for cesarean delivery due to concern for vWD (2).There were no noted adverse anesthetic outcomes, including neuraxial hematoma. There were 8 cases of postpartum hemorrhage (PPH), 50% requiring transfusion. Two patients received DDAVP after PPH onset. PPH etiologies were atony (4 cases), placenta accreta (2), and not defined (2).
To our knowledge, this is the largest case series describing the anesthetic management and outcomes of parturients with vWD. Type 1 vWD is the most common subtype, and neuraxial anesthesia was safely performed in such cases due to procoagulant upregulation in pregnancy. Most patients with vWD in pregnancy were evaluated by a hematologist at our center. Knowledge of the specific subtype of vWD by testing prior to pregnancy was critical to direct management, as was bleeding history and family history. Management must be individualized based on patient characteristics, hematology recommendations, and obstetric delivery plans.
1. Longo NEJM 2016
2. Butwick J Clin Anesth 2007
3. Marrache IJOA 2007
4. Choi AA 2009