Heart transplant in a factor VIII-deficient patient with a high-titre inhibitor: perioperative management using high-dose continuous infusion factor VIII and recombinant factor VIIa

Four years prior to transplantation, a 14-year-old boy with severe haemophi lia A and a high-responding factor VIII (FVIII) inhibitor developed an ante roseptal myocardial infarct while receiving high doses of an activated prot hrombin complex concentrate (PCC). Cardiac transplantation was required for survival because of the ensuing cardiomyopathy. At surgery, the patient's inhibitor titre was 1.8 Bethesda units (BU). High-dose bolus therapy, follo wed by a continuous infusion of FVIII provided excellent operative and init ial postoperative haemostasis without additional blood-product support. Onc e anamnaesis developed on day 6 postoperatively, recombinant factor VIIa (r FVIIa) therapy was initiated. Haemostasis remained excellent, except for th e transient increase in chest-tube bleeding that was noted on clay 7. epsil on -aminocaproic acid was added and haemostasis was re-established. On day 15, rFVIIa was replaced with alternate day infusions of prothrombin complex concentrates (PCCs). On day 21 following the transplant, the patient was d ischarged, remaining on daily FVIII immune tolerance and thrice-weekly PCC prophylaxis. He remains well 24 months after transplant with an inhibitor t itre of 39 BU. This paper describes the second case of cardiac transplantat ion complicated by haemophilia and an inhibitor, and discusses preoperative planning and operative and postsurgical haemostasis management.