DISCUSSION
Heart valve surgery in patients with severe hemophilia remains a
significant challenge due to severe changes in the hemostatic system and
increased risk of bleeding complications. There are no guidelines for
the perioperative management of hemophiliacs undergoing cardiac surgery.
Current practice is based on expert opinions, case reports, or small
case series. In most cases, biological prostheses are used for valve
replacement in hemophiliacs. Since life expectancy of patients with
hemophilia has increased, the possibility of degeneration of biological
prostheses and need for further surgery may carry an even greater risk
to the patient. In the case of mitral insufficiency in young patients,
valve repair is preferable to valve replacement as it potentially avoids
the need for short‐term anticoagulation treatment. The literature
presents a few cases of mitral valve reconstruction in hemophilia A
patients.4-7 Only one describes a minimally invasive
mitral valve repair with concomitant atrium ablation.8Minimally invasive approach for mitral
valve surgery has advantages over standard sternotomy, which may be
especially important in patients with bleeding disorders. It reduces
intra- and postoperative bleeding resulting in a decreased need for
transfusion. Furthermore, ventilation time and ICU or hospital stays are
shortened.9,10
Factor replacement therapy is crucial for successful surgical treatment
of hemophiliac patients. Patients with a pre-planned coagulation
management strategy can successfully undergo complex cardiac surgery as
patients without hemostatic disturbances. According to the
recommendations of the World Hemophilia Federation,11the following FVIII activity levels should be achieved in patients
undergoing major surgical procedures: before surgery, 80–100%;
postoperative days 1–3, 60–80%; postoperative days 4–6, 40–60%;
and postoperative days 7–14, 30–50%. Other recommendations for
patients undergoing coronary artery bypass grafting suggest that FVIII
levels should be 80–100% until postoperative day 5 and above 50%
after that.12 In our case, we maintained FVIII levels
above 80-100% during the first 3 days post-surgery and 50-100% until
postoperative day 7. This strategy allowed us to prevent hemorrhagic
complications in the postoperative period.
Although patients with hemophilia are relatively protected from
thrombosis, there is still a risk of thromboembolism, especially in the
presence of concomitant AF. According to frequently cited antithrombotic
treatment algorithms for hemophiliacs with nonvalvular AF,
antithrombotic regimens are based on baseline FVIII levels and risk of
thromboembolic complications.13 Low-dose aspirin
therapy is indicated in patients with high stroke risk (CHA₂DS₂-VASc
Score ≥2) with baseline FVIII levels of 1–10% and patients with
baseline FVII of 10–30%. Long-term anticoagulation treatment with
vitamin K antagonists is indicated when factor levels are above 30%. In
the presence of AF in hemophiliacs undergoing cardiac surgery,
antithrombotic treatment remains unclear. Concomitant ablation and left
atrial appendage closure should be considered to restore the sinus
rhythm, reduce thromboembolic risk, and decrease dosages or not
prescribe anticoagulants and antiplatelets in patients with low stroke
risk.