Cardiac Surgery in Patients with Hemophilia A and B

2 Hemophilia Unit, Chaim Sheba Medical Center, Israel

Background: Advanced treatment for hemophilia patients have increased their life expectancy and those patients may require major invasive cardiac interventions. Cardiac surgery constitutes a major hemostatic challenge because of sternotomy, the need of total heparinization, extracorporal circulation, and mild hypothermia. There is limited data on performing cardiac surgery in patients with hemophilia disorders.

Methods: We reviewed the medical records of 5 patients with hemophilia A (4) and B (1) undergoing major cardiac surgery. All patients underwent a median sternotomy approach with standard cardiopulmonary bypass approach.

RESULTS: The mean age was 63±15, all were males. Logistic EuroScore was 1.5±0.3. The doses of factor VIII/IX were established on the basis of the effect of the treatment on PT and plasma levels of factors and a clinical assessment of the hemostatic efficacy. There was one in hospital mortality due to delayed tamponade on post-operative-day (POD) 14. The patient was discharged on POD 10 and was re-admitted on POD 12. Table 1 shows patients data operative data. None of the patients had myocardial infarction and there were no thromboembolic complication during follow-up.

Conclusion: Major cardiac surgery can be safely performed in hemophilia patients with strict factor VIII/IX levels monitoring, continuous transfusion of factors and involvement of hematology specialists in patients intra and peri-operative care.