Hemostatic management in a child with moderate hemophilia A in the perioperative period using heparinase-added rotational thromboelastometry during open-heart surgery: A case report

Abstract

Hemostatic management is important in the perioperative period of patients with hemophilia A (HA). Herein, we report the case of a patient with HA who successfully underwent open-heart surgery for atrial septal defects under cardiopulmonary bypass (CPB) by monitoring the effects of heparin and factor VIII (FVIII) replacement therapy using rotational thromboelastometry (ROTEM^®) with the in vitro addition of heparinase. The patient was a 9-year-old boy with moderate HA who underwent open-heart surgery for atrial septal defects using CPB. Perioperative management included a preoperative bolus (52 U/kg) and continuous infusion (4.6 U/kg/hr) of rurioctocog alfa. Post-infusion, the patient’s FVIII activity was elevated to 101.8%, and clot time/clot formation time (CT/CFT) of ROTEM^® was 904/456 s. During the CPB management, anticoagulation with heparin was performed, and CT/CFT of heparinase-added ROTEM^® was 606/299 s and FVIII activity was 78.2%, indicating that FVIII replacement treatment sufficiently improved coagulation potential and adequately controlled perioperative hemostasis.