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%.