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A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More

Severe congenital Factor XI (FXI) deficiency (<20% normal activity) can be associated with significant bleeding disorders, and there has been great concern for severe bleeding following cardiac surgery requiring cardiopulmonary bypass (CPB) in this patient population. Over the past four decades r...

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Autores principales: Kazui, Toshinobu, Nielsen, Vance G., Audie, Spencer D., Venkataramani, Rajagopalan M., Bryant, John T., Swenson, Kristin, Ford, Paul M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9027232/
https://www.ncbi.nlm.nih.gov/pubmed/35448094
http://dx.doi.org/10.3390/jcdd9040118
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author Kazui, Toshinobu
Nielsen, Vance G.
Audie, Spencer D.
Venkataramani, Rajagopalan M.
Bryant, John T.
Swenson, Kristin
Ford, Paul M.
author_facet Kazui, Toshinobu
Nielsen, Vance G.
Audie, Spencer D.
Venkataramani, Rajagopalan M.
Bryant, John T.
Swenson, Kristin
Ford, Paul M.
author_sort Kazui, Toshinobu
collection PubMed
description Severe congenital Factor XI (FXI) deficiency (<20% normal activity) can be associated with significant bleeding disorders, and there has been great concern for severe bleeding following cardiac surgery requiring cardiopulmonary bypass (CPB) in this patient population. Over the past four decades remarkably different approaches to this problem have been taken, including the administration of blood volumes of fresh frozen plasma, administration of activated recombinant Factor VII, and diminutive administration of heparin. We describe a case wherein the patient was assessed in the perioperative period with a point-of-care, viscoelastic hemostasis device (ROTEM), with changes in the intrinsic/Factor XII-dependent coagulation pathway determined before, during, and after CPB. Fresh frozen plasma was administered in small amounts (5–7.5 mL/kg) just before surgery began and just before cessation of CPB. Administering fresh frozen plasma to the patient to nearly normalize in vitro ROTEM hemostasis values at times when hemostasis was needed resulted in no important bleeding occurring or need of further transfusion of other blood products. In conclusion, by using small amounts of fresh frozen plasma guided by ROTEM, an evidenced-based, precision medicine approach resulted in optimized patient care and outcome.
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spelling pubmed-90272322022-04-23 A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More Kazui, Toshinobu Nielsen, Vance G. Audie, Spencer D. Venkataramani, Rajagopalan M. Bryant, John T. Swenson, Kristin Ford, Paul M. J Cardiovasc Dev Dis Case Report Severe congenital Factor XI (FXI) deficiency (<20% normal activity) can be associated with significant bleeding disorders, and there has been great concern for severe bleeding following cardiac surgery requiring cardiopulmonary bypass (CPB) in this patient population. Over the past four decades remarkably different approaches to this problem have been taken, including the administration of blood volumes of fresh frozen plasma, administration of activated recombinant Factor VII, and diminutive administration of heparin. We describe a case wherein the patient was assessed in the perioperative period with a point-of-care, viscoelastic hemostasis device (ROTEM), with changes in the intrinsic/Factor XII-dependent coagulation pathway determined before, during, and after CPB. Fresh frozen plasma was administered in small amounts (5–7.5 mL/kg) just before surgery began and just before cessation of CPB. Administering fresh frozen plasma to the patient to nearly normalize in vitro ROTEM hemostasis values at times when hemostasis was needed resulted in no important bleeding occurring or need of further transfusion of other blood products. In conclusion, by using small amounts of fresh frozen plasma guided by ROTEM, an evidenced-based, precision medicine approach resulted in optimized patient care and outcome. MDPI 2022-04-15 /pmc/articles/PMC9027232/ /pubmed/35448094 http://dx.doi.org/10.3390/jcdd9040118 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Kazui, Toshinobu
Nielsen, Vance G.
Audie, Spencer D.
Venkataramani, Rajagopalan M.
Bryant, John T.
Swenson, Kristin
Ford, Paul M.
A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More
title A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More
title_full A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More
title_fullStr A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More
title_full_unstemmed A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More
title_short A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More
title_sort case report of severe factor xi deficiency during cardiac surgery: less can be more
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9027232/
https://www.ncbi.nlm.nih.gov/pubmed/35448094
http://dx.doi.org/10.3390/jcdd9040118
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