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Type 1 von Willebrand Disease: Factor levels don’t always increase during pregnancy
Abstract Number: SAT-67
Abstract Type: Case Report/Case Series
Von Willebrand Disease (vWD) is the most common inherited bleeding disorder. Over 100 type 1 vWD mutations have been identified, and the phenotypic presentation of type 1 vWD is highly variable with bleeding severity not necessarily predicted by von Willebrand factor (vWF) levels(1). Factor VIII and vWF levels generally increase during pregnancy to normal/supranormal levels, such that type 1 vWD patients rarely require intrapartum treatment with factor replacement and neuraxial analgesia for labor is considered safe(2,3). We describe the intrapartum management of 2 cases of type 1 vWD where vWF and factor VIII activity did not rise during pregnancy.
A 30 yo G3P1011 with gHTN, morbid obesity, lupus and type 1 vWD presented at 37w for repeat cesarean, desiring neuraxial anesthesia. Previous workup revealed she was a DDAVP nonresponder. During both this and her last pregnancy she had no appreciable increase in vWF activity (20% at baseline, 24% at 35w) or factor VIII activity (43% at baseline, 51% at 35w). Hematology recommended Humate 60units/kg and aminocaproic acid 4g loading dose 30 minutes prior to surgery. They stated her factor levels would be adequately corrected with this dosing and spinal anesthesia carried a low risk if placed within 30 minutes of factor replacement. Spinal anesthesia was induced with one attempt and cesarean delivery was uncomplicated. She was maintained on Humate infusions and oral tranexamic acid for 2 weeks post partum. A 28 yo G4P1021 was diagnosed with type 1 vWD at 15w gestation during workup for recurrent epistaxis. She had a term delivery 3 years prior at a birth center, and described mild PPH that did not necessitate transfusion. Throughout pregnancy there was no appreciable increase in vWF activity (12% at baseline, 15% at 37w) or factor VIII activity (47% at baseline, 60% at 37w). She presented for induction at 40w1d. Per Hematology recommendations, Humate 60units/kg was given at the onset of active labor. Epidural anesthesia was not offered; it was felt a Tuohy needle and indwelling catheter posed too high a risk of bleeding even if timing of catheter placement and removal could be coordinated with Humate dosing. She had an uncomplicated vaginal delivery and used nitrous oxide for labor analgesia.
When the quantitative deficit of vWF corrects during pregnancy, the risk of intrapartum bleeding is similar to patients without vWD. It is important to consider that type 1 vWD has high genetic and phenotypic variability. Factor levels should be reassessed throughout pregnancy to identify cases where replacement may prevent complications of hemorrhage and to counsel patients on the risks of neuraxial anesthesia.
1. Reynen, J. Semin thromb hemost. 2016;42:717-723.
2. Butwick, A. J clin anesth. 2007;19:230-233.
3. Marrache, D. Int J Obstet Anesth. 2007;16:231-235.