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Anesthetic Management of a Gravid Patient with Von Willebrand Disease
Abstract Number: T-75
Abstract Type: Case Report/Case Series
Introduction: Patients with coagulopathies often present a challenge when pregnant; their labor and delivery anesthetic plan necessitates advanced planning. We present the case of a patient with HbC trait and Von Willebrand Disease (VWD), whose labor and delivery course went smoothly thanks to a thorough discussion and meticulous plan established between the obstetric and hematology teams.
Case report: A 17 year old, G1P0 patient with HbC trait, VWD (inherited from her father) was followed by the obstetric team during her pregnancy. PMHx also included obesity (BMI 32) and was significant for multiple episodes of prolonged epistaxis. Given her VWD, patient was referred to both the Maternal Fetal Medicine and Hematology. Further testing at 36 weeks revealed a Ristocetin cofactor activity of 20% (normal range 50-150%) and von Willebrand factor (vWF) antigen of 31%. These findings along with a normal Multimer report were suggestive of VWD Type I. Given the patient’s inadequate vWF activity or antigen response to pregnancy, it was recommended she take vWF/Factor VIII concentrate 40 IU/kg (~3200 IU) as a loading dose during labor, followed by ~1600 IU every 12-24 hours for 3-5 days post-delivery. A daily vWF assessment was necessary to ensure that the vWF level did not dip below 50%.
When the patient presented to the L&D Floor at 40w1d, her vWF was measured at 22% and she was started on vWF/Factor VIII concentrate as planned. Given her increased risk of epidural hematoma, she was not a candidate for neuraxial anesthesia and was placed on a low dose IV Fentanyl PCA for analgesia. Her physical exam was significant for a MP class I airway and a thyromental distance > 3 cm. The patient was agreeable to a general anesthetic approach if necessary. To assess the patient’s response to vWF/Factor VIII concentrate, a vWF panel was drawn daily (desired vWF activity level and factor VIII level is >50%). It was necessary to ensure the vWF was not decreased nor elevated since factor VIII levels above 100% increase the risk for thromboembolism. Finally, as vWF levels decline very quickly in the postpartum period, patient needed DDAVP for 3 weeks postpartum (typical range for bleeding is 10-23 days). Her inpatient course was complicated by fever, tachycardia and significant Hgb drop (10 8.2) without clinical signs of bleeding. She received 2 uPRBC, and was started on oral tranexamic acid (650mg q12h). Patient subsequently remained stable, and infusions of DDAVP were continued as an outpatient, with monitoring of Factor VIII levels.
Conclusions: Gravid patients with VWD need to be evaluated carefully prior to delivery, and their care must be discussed in a multidisciplinary setting. Repletion of vWF and Factor VIII must be done cautiously, with frequent laboratory assessments to prevent both hemorrhage and thromboembolism.
James et al Haemophilia. 2015 Jan;21(1):81-7. doi: 10.1111/hae.12568.