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An unanticipated prolonged baseline ACT during cardiac surgery due to factor XII deficiency


1 Department of Anesthesiology and Pain Management, Maastricht Universitair Medisch Centrum, P. Debyelaan 25, Maastricht, The Netherlands
2 Department of Cardiothoracic Surgery, Maastricht Universitair Medisch Centrum, P. Debyelaan 25, Maastricht, The Netherlands

Correspondence Address:
Heleen J.C L. Apostel
Department of Anesthesiology and Pain Management, Maastricht Universitair Medisch Centrum, P. Debyelaan 25, Maastricht - 6229 HX
The Netherlands
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aca.aca_255_20

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Year : 2022  |  Volume : 25  |  Issue : 2  |  Page : 229-232

 

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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.






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1 Department of Anesthesiology and Pain Management, Maastricht Universitair Medisch Centrum, P. Debyelaan 25, Maastricht, The Netherlands
2 Department of Cardiothoracic Surgery, Maastricht Universitair Medisch Centrum, P. Debyelaan 25, Maastricht, The Netherlands

Correspondence Address:
Heleen J.C L. Apostel
Department of Anesthesiology and Pain Management, Maastricht Universitair Medisch Centrum, P. Debyelaan 25, Maastricht - 6229 HX
The Netherlands
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aca.aca_255_20

Rights and Permissions

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.






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