Peri-cardiac surgery coagulation management in a severe hemophilia A patient: A case report

Hemophilia A (HA) is an X-linked recessive disorder caused by clotting factor VIII (FVIII) deficiency. There is limited data on the use of replacement therapy in cardiac surgery. Since no international guideline for anticoagulation in such patient exists, careful thought should be taken to design an individualized anticoagulation strategy.

We report a 54-year-old male with severe HA with FVIII activity of 0.8% when he was first diagnosed, who underwent successful mitral valve repair and coronary artery bypass graft with FVIII replacement perioperatively.

Transthoracic echocardiography and coronary angiography confirmed the HA patient with the diagnosis of severe mitral valve regurgitation and left anterior descending artery stenosis.

Before surgery, a bolus of 1000 IU FVIII was injected, which obtained an FVIII of 80%. After induction, a 3750 IU bolus of FVIII was injected and subsequent FVIII level reached 135%. Mitral valve repair and coronary artery bypass graft with FVIII replacement were performed. After the surgery, a repeat FVIII activity level was 50.6%. The 400 mL of autologous blood and 700 mL of cardiopulmonary bypass (CPB) machine blood was returned to the patient as well as 4 units of fresh frozen plasma with an additional bolus of 1000 IU FVIII. 100 mg aspirin per day alone was given after surgery.

The patient recovered uneventfully and 1-year follow-up showed no complications.

The anticoagulant or antiplatelet regimen of HA patient following surgery should be individualized based on the evaluation of the risk factors for bleeding and thrombosis and the lowest FVIII activity ever recorded after FVIII replacement therapy.