Successful Management of an Obstetric Patient with von Willebrand Disease Type 2N and Malignant Hyperthermia
Abstract Number: F-64
Abstract Type: Case Report/Case Series
Introduction: Von Willebrand Disease (VWD) is the most common inherited coagulopathy affecting women in pregnancy. VWD may result from quantitative deficiencies or qualitative defects in von Willebrand Factor (VWF) and it is important to consider the subtype of VWD because it will affect peripartum management. Patients with VWD type 2N, characterized by mutations at the VWF binding site for factor VIII, have significantly reduced circulating half-life of factor VIII. This case presents the peripartum management of a parturient with VWD type 2N.
Case: A 27 year old G1P0 patient presenting at 38+3 weeks gestation with a history significant for VWD type 2N, an allergy to desmopressin, and malignant hyperthermia (MH). A scheduled induction was performed secondary to VWD type 2N and MH so that the patients coagulopathy could be optimized and appropriate facilities were available in case of emergency. Hematology consultation was obtained at 24 weeks gestation to guide further testing & provide recommendations for peripartum management. Labs drawn at 26 & 33 weeks gestation showed marked deficiency of factor VIII (9% & 20%, respectively) consistent with her established diagnosis of VWD type 2N. A dose of VWF/Factor VIII 40 U/kg was administered prior to induction of labor and continued every 12 hours for 14 days postpartum. Labor epidural placed without complication. Her pain was well-controlled for approximately 22 hours at which point the obstetric team recommended a Cesarean section (C-section) secondary to failure to progress. The patient was given an additional VWF/Factor VIII 20U/kg & the epidural was removed in favor of a spinal anesthetic for C-section. C-section was performed with estimated blood loss of 1000mL & postpartum vaginal bleeding was not judged to be excessive. All PTT & Factor VIII values checked while the patient was in L&D were found to be in normal ranges, indicating accurate replacement therapy.
Discussion: Successful management for cesarean & vaginal deliveries with VWD type 2N has been rarely reported in literature. (1-5) Neuraxial anesthesia can be safely accomplished in VWD type 2N with proper replacement therapy. In patients with VWD type 2N and persistently low factor VIII levels at term, VWF/Factor VIII concentrates are likely more effective than desmopressin (1,6) given the presence of normal VWF in circulation is necessary to normalize the half-life & plasma concentrations of factor VIII. (1)
1. Nishino, M. et al. (1996) International Journal of Hematology 64:127-134.
2. Watanabe, T. et al. (1997) Obstetrics and Gynecology 89(5 Pt 2):859.
3. Dennis, M. et al. (2000) Haemophilia 6:696-697.
4. Dell’Edera, D. et al. (2011) Journal of Biological Regulators and Homeostatic Agents 25(3):469-475.
5. Castaman, G. et al. (2005) Journal of Thrombosis and Haemostasis 3(2):391-392.
6. Mazurier, C. et al. (1994) British Journal of Haemotology 88(4):849-854.