CLINICAL CASE
A 50-year-old male (62 kg body weight) with a history of hemophilia A entered our clinic with complaints of dyspnea during exercise, palpitations, and weakness. The FVIII level was 0.7% when hemophilia was first diagnosed. The patient had mitral valve prolapse with moderate regurgitation for over 12 years. A persistent form of AF was diagnosed a year prior to presentation at our clinic for which he received bisoprolol (2.5 mg/day).
Upon admission, the patient had deformation and limited mobility in the elbow and knee joints (due to recurrent hemarthrosis), apex systolic murmur, and an abnormal heart rhythm with a heart rate ranging from 55 to 160 beats per minute (bpm). An ECG revealed AF with a ventricular rate of 110 bpm. Transthoracic echocardiography (TTE) showed both mitral valve leaflets prolapse with severe regurgitation (vena contracta of 0.8 cm, effective regurgitant orifice area of 45 mm2), significant enlargement of the left atrium (volume of 270 mL), left ventricular diastolic volume of 170 mL, and ejection fraction of 50%. The FVIII level was 8.7% after replacement therapy with 1000 IU FVIII (16 IU/kg) in 1 day. Inhibitory antibodies toward FVIII were not detected. CHA₂DS₂-VASc Score was 1.
A perioperative coagulation management strategy for this patient was developed. Thirty minutes preoperatively, he was administered 4000 IU (65 IU/kg) of FVIII concentrate and reached a 109% level of FVIII activity. Surgery was performed using a right anterolateral minithoracotomy (approximately 5 cm long) in the fifth intercostal space with femoral artery and vein cannulation. Tranexamic acid (1 g/hour) was infused, beginning with the skin incision to the completion of the procedure. Prior to cannulation, a heparin bolus of 300 IU/kg was administered and resulted in an activated clotting time ≥ 400 s while on cardiopulmonary bypass, during which FVIII replacement therapy was not performed. Myocardial protection was performed using antegrade cardioplegia (Custodiol, Dr. F Kӧhler Chemie, Bensheim, Germany). The mitral valve was repaired by quadrangular resection of the posterior leaflet with sliding plasty and decalcification of the posterior annulus, and implantation of a flexible band (40 mm, CardioMed, Penza, Russia) (Figure 1).
The left and right atrial ablation was performed according to the Cox maze IV pattern3 using the nitrous oxide-based CryoICE Probe (AtriCure Inc., Mason, OH, USA). The left atrial appendage was excluded using a double-layer 3/0 polypropylene suture. After weaning the patient from bypass, protamine was administered to inactivate heparin. The cardiopulmonary bypass and cross-clamping times were 110 and 90 min, respectively. After inactivating heparin, 2000 IU (32 IU/kg) of FVIII was injected (Figure 2).
The patient was extubated 10 hours after being transferred to the intensive care unit (ICU). The early postoperative period was complicated by moderate heart failure; therefore, the patient received inotropic therapy during the first day. An episode of supraventricular tachycardia was noted in the ICU and cured by amiodarone infusion. The total drainage losses were 700 mL (first day), and 400 mL (second day). The patient received 500 mL of fresh frozen plasma on postoperative day 1. The minimum hemoglobin level was 89 g/L and blood transfusions were not required. The patient was transferred from the ICU to the general ward on postoperative day 3. Postoperatively, no anticoagulant or antiplatelet therapy was prescribed.
Postoperative replacement therapy was maintained as follows. A bolus of 3000 IU (48 IU/kg) FVIII was administered 12 hours after the first injection, then 3000 IU of FVIII was continued every 12 hours on days 1–3, 2000 IU (32 IU/kg) every 12 hours on days 4–7, and 3000 IU (48 IU/kg) once per day on days 8–14. Beginning on postoperative day 15, 1000 IU FVIII (16 IU/kg) was administered every other day. The FVIII levels were measured twice a day during the first 3 days post-surgery and once every day until discharge.
TTE at the time of discharge revealed trivial mitral regurgitation. A 24 h Holter monitoring device confirmed a sinus rhythm with an average daily heart rate of 72 bpm and freedom from AF. The patient was discharged 15 days after surgery. The hemoglobin level at discharge was 119 g/L. Bisoprolol and amiodarone (2.5 and 200 mg/day, respectively) were prescribed for 3 months.
Two years post-surgery, the patient was classified with New York Heart Association class I heart failure, had sinus rhythm without AF paroxysms, and had no bleeding or thrombotic complications. TTE revealed trivial mitral insufficiency and a left ventricle ejection fraction of 60%.