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Anesthetic Management for a Successful Pregnancy in a Patient with Severe Congenital Hypofibrinogenemia and Prior Fetal Demise
Abstract Number: F3C-3
Abstract Type: Case Report/Case Series
Introduction: Congenital afibrinogenemia or severe hypofibrinogenemia predisposes patients to spontaneous abortions, placental abruption, obstetric hemorrhage, and thrombosis (1). Few reports detail the peripartum anesthetic and obstetrical management of these patients.
Description: We describe a patient with severe congenital hypofibrinogenemia (baseline fibrinogen activity less than 30 mg/dL) who became pregnant 6 months after a prior intrauterine fetal demise (IUFD) at 36 weeks gestation. She received fibrinogen concentrate (doses between 4300 and 5500 mg) two to three times/week since 4 weeks gestation to prevent fetal loss associated with this condition. To limit the possibility of recurrent IUFD, she was admitted at 32 weeks for monitoring with a plan for induction of labor (IOL) at 36 weeks. At 34+4 weeks, she was induced due to concerns over fetal heart rate changes.
A baseline thromboelastogram (TEG) was normal (2). A radial arterial line was placed, and a baseline fibrinogen level was 192 mg/dL. We started a continuous infusion of fibrinogen concentrate (Figure 1), with a target of ≥150 mg/dL during latent labor and for neuraxial blockade/catheter removal. A target of ≥200 mg/dL was set during active labor. She underwent uncomplicated epidural catheter placement and spontaneous vaginal delivery of a live infant, with a 300 mL estimated blood loss. The epidural catheter was removed 5 hrs later; her fibrinogen level was 168 mg/dL. The fibrinogen concentrate infusion was discontinued 42 hours post delivery. No significant postpartum complications occurred, and she was discharged on postpartum day 4.
Discussion: Close monitoring of plasma fibrinogen levels, careful titration of fibrinogen concentrate, and TEG data confirming normal hemostasis helped facilitate uncomplicated neuraxial labor analgesia. Expectant management and careful monitoring contributed to a vaginal delivery of a live infant without significant postpartum hemorrhage. To help determine a safe fibrinogen threshold for performing neuraxial blockade and minimize postpartum hemorrhage, we recommend that a registry be established to report interventions and outcomes for women with rare disorders of the coagulation system.
1. Ann Hematol. 2007;86(9):693-4.
2. Anaesthesia. 2012;67(7):741-7.
3. Clin Exp Pharmacol Physiol. 2016;43(2):149-56.