Severe Congenital Factor VII Deficiency with Normal Perioperative Coagulation Profile Based on ROTEM Analysis in a Hepatectomy

Abstract

Patient: Female, 21-year-old Final Diagnosis: Congenital factor VII deficiency Symptoms: Coagulopathy • radial artery thrombosis Medication: — Clinical Procedure: — Specialty: Anesthesiology • Hematology Objective: Rare disease Background: Factor VII (FVII) deficiency is the most common autosomal-recessive bleeding disorder. FVII activity level (FVII: C) of 10-20% is often used as the threshold for administering activated recombinant FVII (rFVIIa) for patients undergoing major surgery. However, rFVIIa is expensive and carries the risk of a thromboembolic event, and thus should only be administered when truly indicated. Case Report: A 22-year-old woman with 8% FVII: C underwent a hepatectomy. Although there were no clinical signs of bleeding, peri-operative administration of rFVIIa was recommended by the hematologist (first dose at surgical incision, then 4 h later, then every 12 h until 48 h postoperatively). Intraoperatively, serials of ROTEM analysis were performed to evaluate the effect of rFVIIa administration. No significant effect of rFVIIa was seen on NATEM. Surgery was unremarkable, without any significant blood loss. The patient developed radial artery thrombosis 24 h postoperatively, the arterial line was removed, and rFVIIa was discontinued (PT: 14.6, FVII: C 36%). On POD 3, INR was elevated (3.15, FVII: C 3%). To correct INR, the patient was transfused 8 units of FFP, despite any signs of clinical bleeding. However, INR and FVII: C did not correct and the patient was discharged on POD 7 in a stable condition. Conclusions: Even with FVII: C of 8%, the ROTEM analysis revealed a normal coagulation status. The administration of rFVIIa did not improve the already normal baseline coagulation profile, but rather potentially led to an accelerated coagulation or hypercoagulable state and may have led to the radial artery thrombosis. We endorse the use of viscoelastic testing for hemostasis assessment and factor replacement in congenital FVII deficiency.

A 36-Year-Old Woman with Acute Liver Failure Following Acetaminophen Overdose, Raised INR of 8.7, and Normal Blood Viscosity Measured by Rotational Thromboelastometry (ROTEM)

“…Dotsch et al reported that ROTEM was a better predictor of postoperative bleeding than plasma fibrinogen concentration [ 30 ]. Multiple studies found that thromboelastography-guided transfusion decreased FFP transfusions and intraoperative blood loss and was superior to conventional coagulation tests [ 31 – 34 ]. The International Liver Transplantation Society (ILTS) ERAS4OLT.org Consensus…”

The Non-Activated Thromboelastometry (NATEM) Assay’s Application among Adults and Neonatal/Pediatric Population: A Systematic Review

“…A total of 30 NATEM articles (1477 participants) from 2010 to 2022 were included. Twenty studies were conducted in Europe [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32], three studies were conducted in Japan [33][34][35] and the USA [36][37][38], one study in Australia [39], one in Russia [40], one in Brazil [41] and one study was conducted in Israel [42]. The outcomes from the 30 included studies were grouped into four categories: experimental study using NATEM assay (n = 7), evaluation of NATEM assay in healthy human volunteers (n = 8), in patients versus healthy volunteers (n = 7) and in patients only (n = 8).…”

“…Rapid transport in pneumatic systems may influence the reliability of results by platelet activation and/or contact activation, as a NATEM assay revealed it was expressed with a shortened CT, which reflects the initiation of clot formation. Ultimately, Yeom et al [38] aimed to examine the use of thromboelastometry assays in testing coagulation disorders and its contribution in factor replacement in congenital FVII deficiency, and showed that NATEM analysis indicated a normal coagulation condition in a 22-year-old woman with an FVII:C of 8%; however, she received activated recombinant FVII (rFVIIa), which most probably led to thrombosis of the radial artery. Yada et al [33] assessed the coagulation status of seven patients with hemophilia A using NATEM.…”