In the case reported here, the situation was complicated by a congenital deficiency of factor VII (FVII), which increased the risk of surgical and postoperative bleeding. Perioperative rotational thromboelastometry coagulation monitoring avoided empiric prophylactic treatment with recombinant activated FVII (rFVIIa). CASE REPORT A 5-year-old girl weighing 37.8 lb of the JW faith without cardiologic symptoms or a history of bleeding episodes had been diagnosed with ostium secundum atrial septal defect (ASD) by echocardiogram and was scheduled for transcatheter device closure of the defect. The transesophageal echocardiographic study and cardiac catheterization confirmed a 16-mm diameter defect (Qp:Qs ratio of 2.4:1) and contraindicated transcatheter device closure due to insufficient rims and the extension of the defect into the inferior vena cava. The patient was scheduled for ASD closure with CPB. The patient’s parents, also JWs, did not authorize the use of whole blood or blood components (ie, packed red blood cells, fresh frozen plasma, platelets, and cryoprecipitate), but accepted the use of coagulation factor concentrates (ie, fibrinogen, factor VIIa, and prothrombin complex), erythropoietin, intravenous iron, and cell salvage. Preoperative routine tests showed a hemoglobin (Hb) concentration of 12.5 g/dL, hematocrit of 36.6%, a mild iron deficit (iron: 45 g/dL, transferrin: 253 mg/dL, transferrin saturation: 14%, ferritin: 40 ng/mL, and total iron-binding capacity: 321 g/dL), and an abnormal prothrombin time (PT) of 15 seconds (63% activity, international normalized ratio 1.3) with a normal activated partial thromboplastin time. After the confirmation of prolonged PT, further investigations revealed a mild FVII deficiency (43.3% activity), whereas all other coagulation factors were within the normal range. Surgery was scheduled 13 days later to allow adequate preparation of the patient and to avoid the administration of blood products during the procedure. Treatment was initiated with a short preoperative course of 7 doses of subcutaneous human recombinant erythropoietin (r-HuEPO) at a dosage of 600 U/kg every other day. At the start of erythropoietin treatment, the maximum single intravenous dose of ferric carboxymaltose (255 mg) was administered in a 15-minute infusion to prevent relative iron deficiency. The patient also received oral supplements of vitamin B12 and folic acid throughout the preoperative period. A complete blood count and coagulation panel were obtained the day before surgery, showing an increase in Hb of 2 g/dL. The PT remained prolonged because of the FVII deficiency. Laboratory findings from preoperative and postoperative tests are summarized in Table 1. Anesthesia management consisted of induction with sevoflurane in oxygen and air, followed by fentanyl and cisatracurium administration to facilitate endotracheal intubation, and maintenance with remifentanil, 0.25 g/kg/min, and 2% sevoflurane in an oxygen/air mixture. A radial arterial catheter and a right internal jugular central venous catheter were inserted for invasive pressure monitoring. Near-infrared spectroscopy and bispectral index were used for neurologic monitoring. After heparin administration and cannulation, a direct suturing of the edges of the ASD was performed under moderate hypothermic (32°C) CPB (36 minutes) with a cross-clamp time of 18 minutes. After rewarming, modified ultrafiltration, and protamine administration, weaning from CPB was achieved uneventfully using a continuous infusion of low-dose dopamine (3 g/kg/min). Preoperative erythropoietin treatment was combined with fibrinolytic prophylaxis intraoperatively (tranexamic acid given in a bolus dose of 15 mg/kg before surgical incision), a meticulous surgical technique, a crystalloid low-volume priming CPB circuit (500 mL), modified ultrafiltration (500 mL), and a reduction in the number and volume of blood samples for laboratory tests. During and immediately after surgery, coagulation was monitored with rotational thromboelastometry (ROTEM; TEM International, GmbH, Munich, Germany), which made the administration of coagulation factor concentrates unnecessary (Table 2). Bleeding was not heavier than normal. The chest drains were removed 48 hours after surgery with a total volume of 106 mL. The patient was discharged from the pediatric intensive care unit on day 2 after the surgery and was sent home on day 6. No transfusions of blood products were administered.
Read full abstract