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
S. Sheth et al., 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, HAEMOPHILIA, 7(2), 2001, pp. 227-232
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.