Haemophilia A is an X-linked congenital bleeding disorder caused by mutations in coagulation factor VIII gene (F8) which result in absent, deficient, or defective factor VIII protein (FVIII). The range of clinical severity is proportional to the plasma level of FVIII. A FVIII level of less than 1% produces severe disease. Patients with severe disease have spontaneous bleeding in joints, soft tissues, and vital organs as well as exaggerated bleeding following trauma or surgery. Preventive replacement therapy (with plasma-derived or recombinant FVIII) has increased the life expectancy of subjects with haemophilia A, but this complex pathology requires expert clinical management by a multidisciplinary team (paediatrician, haematologist, orthopaedist, etc.)1,2. Cardiac surgery in infants with haemophilia can be a particularly challenging problem because significant coagulation abnormalities may occur during and after surgery. Careful pre-operative planning and meticulous post-operative monitoring of FVIII replacement therapy are essential for maintaining optimal haemostasis. Although considerable advances have been made in the management of haemophilia, there is a lack of guidelines pertaining to cardiac surgery in children.
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