Congenital factor XII deficiency is a rare condition. We report a case of a
ortic valve replacement (AVR) in a 63-year-old man with factor XII deficien
cy. On admission, the patient's activated partial thromboplastin time (aPTT
) was prolonged (271 s), and activated clotting time was 500 s. His factor
XII level was < 3 %. The Sonoclot (R) signature showed an abnormal pattern.
AVR with a prosthetic valve (St. Jude Medical) was performed safely after
the normalization of aPTT and the Sonoclot signature by frozen plasma trans
fusion. The perioperative management in patients with factor XII deficiency
is discussed.