Factor XI deficiency and aortic valve replacement: Perioperative management

Asian Cardiovasc Thorac Ann. 2017 Jul;25(6):450-452. doi: 10.1177/0218492316644075. Epub 2016 Apr 18.

Abstract

Severe factor XI deficiency (hemophilia C) is a rare coagulation disorder. A 73-year-old woman, a homozygote for factor XI deficiency, required aortic valve replacement. An initial dose of 15 U kg-1 of factor XI concentrate was administered preoperatively and on postoperative day 3. During surgery, concentrated red cells, fresh frozen plasma, platelets, tranexamic acid, and fibrinogen were transfused. Intraoperative bleeding and total chest drainage were minimal. Postoperatively, there was no need for further transfusions and no bleeding or thrombotic complications occurred. The patient was well 16 months after surgery.

Keywords: Aortic valve insufficiency; Blood loss; Factor XI deficiency; Heart valve prosthesis implantation; surgical.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antifibrinolytic Agents / administration & dosage*
  • Aortic Valve / surgery*
  • Blood Coagulation / drug effects*
  • Blood Component Transfusion*
  • Erythrocyte Transfusion
  • Factor XI Deficiency / blood
  • Factor XI Deficiency / diagnosis
  • Factor XI Deficiency / therapy*
  • Female
  • Fibrinogen / administration & dosage*
  • Heart Valve Prosthesis Implantation* / adverse effects
  • Humans
  • Intraoperative Care
  • Plasma
  • Platelet Transfusion
  • Tranexamic Acid / administration & dosage*
  • Treatment Outcome

Substances

  • Antifibrinolytic Agents
  • Tranexamic Acid
  • Fibrinogen