TY - JOUR
T1 - An unanticipated prolonged baseline ACT during cardiac surgery due to factor XII deficiency
AU - L Apostel, Heleen J C
AU - De Bie, Ben
AU - Kats, Suzanne
AU - Schreiber, Jan-Uwe
N1 - Publisher Copyright:
© 2022 Wolters Kluwer Medknow Publications. All rights reserved.
PY - 2022/4/15
Y1 - 2022/4/15
N2 - Factor XII (FXII) deficiency is a congenital disorder inherited as an autosomal recessive condition. In his heterozygous form, it is relatively common in the general population. However, a total absence of FXII as seen in homozygous patients, is rare, with an incidence of approximately 1/1,000,000 individuals. Surprisingly, FXII deficiency is rather associated with thromboembolic complications. Patients do not experience a higher risk of surgical bleeding despite a markedly prolonged activated partial thromboplastin time. Given its low incidence in the general population, the finding of an unknown FXII deficiency is rare during cardiac surgery. This unique case describes a patient with an unanticipated prolonged baseline activated clotting time (ACT) during cardiac surgery in which his bleeding history and rotational thromboelastometry tracings lead us to the diagnosis of a FXII deficiency. The finding of a hypocoagulable INTEM tracing and a concurrent normal EXTEM tracing in a sample of a patient with prolonged ACT and adverse anamnestic bleeding history should prompt clinicians to consider a FXII deficiency. It may help clinicians in further perioperative management where there is not enough time to wait for the results of individual coagulation factor testing.
AB - Factor XII (FXII) deficiency is a congenital disorder inherited as an autosomal recessive condition. In his heterozygous form, it is relatively common in the general population. However, a total absence of FXII as seen in homozygous patients, is rare, with an incidence of approximately 1/1,000,000 individuals. Surprisingly, FXII deficiency is rather associated with thromboembolic complications. Patients do not experience a higher risk of surgical bleeding despite a markedly prolonged activated partial thromboplastin time. Given its low incidence in the general population, the finding of an unknown FXII deficiency is rare during cardiac surgery. This unique case describes a patient with an unanticipated prolonged baseline activated clotting time (ACT) during cardiac surgery in which his bleeding history and rotational thromboelastometry tracings lead us to the diagnosis of a FXII deficiency. The finding of a hypocoagulable INTEM tracing and a concurrent normal EXTEM tracing in a sample of a patient with prolonged ACT and adverse anamnestic bleeding history should prompt clinicians to consider a FXII deficiency. It may help clinicians in further perioperative management where there is not enough time to wait for the results of individual coagulation factor testing.
KW - Cardiac Surgical Procedures
KW - Factor XII
KW - Factor XII Deficiency/complications
KW - Humans
KW - Partial Thromboplastin Time
U2 - 10.4103/aca.aca_255_20
DO - 10.4103/aca.aca_255_20
M3 - Article
C2 - 35417978
SN - 0971-9784
VL - 25
SP - 229
EP - 232
JO - Annals of Cardiac Anaesthesia
JF - Annals of Cardiac Anaesthesia
IS - 2
ER -