Twin Delivery in a Parturient with a Complete Absence of Factor X and Minor Factor VII Deficiency
Abstract Number: 150
Abstract Type: Case Report/Case Series
Factor X, a vitamin-K dependent coagulation factor, is the first enzyme in the common pathway of thrombus formation. Inherited Factor X deficiency is a rare, autosomal recessive coagulopathy that occurs in about 1:1,000,000 individuals. Severe Factor X deficiency poses significant challenges in pregnancy as it is associated with recurrent miscarriage, uterine bleeding, and premature labor. Anesthetic management of these patients in the peripartum period can be challenging as coagulopathy can be a contraindication to neuraxial anesthesia and if severe bleeding occurs, hemodynamic instability may result.
We report the case of a 26 year-old nulliparous female with twin gestation and severe Factor X and mild Factor VII deficiency who presented in preterm labor with a PT >138, INR >19.6, and PTT>123.9. Her pregnancy was a result of in vitro fertilization and she had had 11 miscarriages during previous attempts to conceive. This pregnancy was complicated by a 7-cm subchorionic hematoma at 8 weeks gestation requiring weekly transfusions of FFP; previously, she had required FFP transfusions to control hemorrhage during menses.
Because the 33-week twins were vertex-vertex on admission, she was a candidate for vaginal delivery should labor progress despite tocolysis. She was placed on a transfusion regimen of FFP to normalize her coagulopathy. Despite this therapy, her labor progressed and her INR remained above 1.5. She was prepared for an attempted vaginal delivery with intravenous analgesia since she was not a candidate for neuraxial anesthesia.
After a lengthy 2nd stage of labor, Twin A was delivered spontaneously and Twin B, via a forceps-assisted delivery. She received a total of 8 units of FFP in the peripartum period and no bleeding complications were encountered. Her peripartum course was complicated by dyspnea and hypoxemia, with a differential diagnosis including fluid overload, pneumonia, pulmonary hemorrhage, amniotic fluid embolism, TACO (transfusion associated circulatory overload), and TRALI (transfusion related acute lung injury). Supportive therapy was continued with oxygen and diuresis and empiric antibiotics were started. The patient was weaned off of supplemental oxygen in 24 hours and was discharged home in stable condition on postpartum day 4.
While improvement of coagulation abnormalities with blood products may decrease the risk and severity of bleeding, aggressive transfusion is not without consequence. Alternative therapies, such as recombinant factors including Factor VIIa and prothrombin complex concentrates, should be considered to decrease the complications of volume overload, TACO, and TRALI associated with FFP transfusion. This case highlights the challenges encountered when managing patients with severe coagulopathy in the peripartum period and emphasizes the need for a multidisciplinary team approach to care.