Successful management of maternal factor VII deficiency in a cesarean section

Abstract

Factor VII (FVII) deficiency is an infrequent hereditary bleeding disorder that can make excessive bleeding in surgical interventions, such as a postpartum hemorrhage in a cesarean section. Although a recombinant form of activated FVII has been applied for bleeding control in FVII-deficient patients, its applications in the field of obstetrics are still limited, especially in Korea. Replacement of blood products is still preferred as first-line therapy, with antifibrinolytic agents used as adjunctive therapy. We report herein the case of a successful cesarean section in an 18-year-old woman with FVII deficiency.

Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review

“…Hemostatic treatments used before general anesthesia comprised TXA (three cases), 16,17 FFP (two cases), 17,55 platelet infusion (two cases), 17,48 and rFVIIa (one case), 16 including combinations of these treatments 16,17 . Remifentanil, a short‐acting synthetic opioid analgesic, was used intravenously for patient‐controlled analgesia for two vaginal deliveries (one woman with severe FVII deficiency and one with non‐severe deficiency); FVII deficiency was a deciding factor in one case 55 …”

“…There were 22 vaginal 11 , 14 , 16 , 18 , 35 , 36 , 40 , 49 , 50 , 53 , 56 , 57 , 58 and 22 caesarean deliveries. 12 , 16 , 17 , 19 , 20 , 23 , 47 , 48 , 51 , 52 , 53 , 54 , 55 Ten emergency caesarean deliveries were performed for reasons including failure to progress or failed induction of labor (six cases), 16 , 47 , 48 , 53 , 54 fetal distress (three cases), 17 , 19 , 20 and breech presentation (one case). 55 Three caesarean deliveries were planned; one to reduce risk of human immunodeficiency virus transmission to the fetus, 51 one due to prior maternal intracranial hemorrhage, 53 and one at maternal request.…”

“…Treatments used included intravenous rFVIIa in 16 cases, 16 , 23 , 35 , 36 , 47 , 49 , 51 , 53 , 56 , 57 plasma‐derived FVII in five cases, 11 , 18 , 20 , 58 and FFP in seven cases. 12 , 14 , 17 , 19 , 20 , 50 , 55 Other prophylactic treatments included oral TXA (four cases) 16 , 17 , 57 and platelet transfusion (three cases). 17 , 48 , 52 …”

Diagnosis and treatment discussion of congenital factor VII deficiency in pregnancy: A case report

“…When the laboratory examination shows prolonged PT with normal APTT or both PT and APTT are normal[ 17 ] (with an exception for the causes of abnormal bleeding and pregnancy failure caused by related diseases), then FVIID should be suspected. The prolonged PT can be corrected by normal plasma[ 18 ], while the decrease in the FVII activity and the detection of the F7 gene[ 2 ] can further confirm congenital FVIID in patients. We summarize the diagnosis process of pregnancy with congenital FVIID in Figure 2 .…”

“…FVII is a determining factor in activating the exogenous coagulation pathway[ 1 ]. Congenital factor VII deficiency (FVIID) is a rare autosomal recessive hemorrhagic disease caused by F7 gene mutation and leads to a decrease in FVII number or functional defect[ 2 ]. The F7 gene is located on chromosome 13 (13q34) and consists of 9 exons and 8 introns, covering a 12.8 kb genome area.…”