A case report of the coagulation management of an adult with moderate haemophilia A undergoing transcatheter aortic valve implantation

Haemophilia A is an X-linked bleeding disorder resulting from factor VIII deficiency. While surgical management requires the correction of factor VIII levels by the administration of factor VIII concentrate, antiplatelet therapy after lower-risk cardiac surgery/procedures (such as transcatheter aortic valve implantation—TAVI) is not yet standardized. This report describes the management of a patient with moderate haemophilia A undergoing TAVI for severe aortic stenosis.

A 76-year-old man with untreated haemophilia A was admitted with traumatic bleeding. After initial stabilization, he developed acute heart failure due to newly discovered severe aortic stenosis. TAVI procedure was performed by transfemoral access according to multidisciplinary team-discussion; a bio-prosthetic valve was successfully implanted. We outline the peri-procedural haemostatic management, which included factor VIII replacement therapy to achieve guideline-recommended activity levels, followed by progressive dose reduction. In view of the high bleeding risk and absence of significant coronary artery disease, antiplatelet therapy was limited to a short course. The procedure was successful, with no bleeding complications.

In our patient, replacement therapy with rFVIII was initiated with a target FVIII activity between 80 and 100 IU/dL. During the procedure, unfractionated heparin was administered to achieve an ACT target 29 of 200 s. Post-operatively, FVIII replacement therapy was maintained above 60 IU/dL for the first 30-72 h, followed by 40-50 IU/dL from day 4 to day 7. Subsequently, it was decided to continue low-dose FVIII replacement for 32 additional 7 days. This strategy led to a successful management of both cardiological and hematological aspects.

Although evidence is limited to case reports, TAVI appears to be feasible and safe in patients with haemophilia A. Maintaining high factor VIII activity levels and minimizing antithrombotic exposure through multidisciplinary evaluation can effectively reduce haemorrhagic risk. Our case, in line with previous reports, resulted in no bleeding complications.