Background: Hemophilia B (HB) is a rare X-linked congenital bleeding disorder characterized by a deficiency of coagulation factor IX (FIX). There is evidence that people with hemophilia develop atherosclerosis at similar rates to those in the general population; moreover, they also may develop ischemic heart disease.

Aims: The aim of this clinical case is to highlight that a good management of HB patients undergoing coronary artery bypass graft (CABG) is essential for successful intervention.

Methods: A 58-year-old man suffering from severe HB (FIX < 1%) reported symptomatic retrosternal burning when walking in cold weather. He presented many comorbidities, as arterial hypertension, type 2 diabetes mellitus, hyperlipidemia, overweight. Coronary angiography highlighted obstructive coronary artery disease with two-vessel critical stenosis.
In consideration of the patient’s ischemic and hemorrhagic risk, CABG without extracorporeal circulation (off-pump, OP) was performed, through skeletonized left internal mammary artery on anterior interventricular artery. 6000 UI of albutrepenonacog, a recombinant FIX fused with albumin, were administered intravenously one hour before the procedure, together with tranexamic acid at 500 mg/hour as a continuous infusion. After OP-CABG, tranexamic acid was administered 1 gram every 8 hours, for seven days. Albutrepenonacog 4000 IU was infused in the third post-surgery day, and, afterwards, once a week to maintain FIX levels above 50%. One unit of concentrated red blood cells was transfused. No hemorrhagic or thrombotic events were documented. Acetylsalicylic acid 75 mg/day was prescribed.

Results: Four months of cardiological rehabilitation were carried out, with progressive subjective improvement and tolerance to effort. After a 6-month follow-up, ischemic heart disease was clinically stable.

Conclusions: This report shows that a good management of coronary artery disease in HB patients is possible. It requires close cooperation between hemophilia and cardiology teams, and should be individualized in terms of hemophilia therapy and choice of the best treatment for the disease.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/off-pump-coronary-artery-bypass-graft-management-in-a-severe-hemophilia-b-patient/