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.