{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T14:55:21Z","timestamp":1791816921176,"version":"4.3.4"},"reference-count":16,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2022,4,15]],"date-time":"2022-04-15T00:00:00Z","timestamp":1649980800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCDD"],"abstract":"<jats:p>Severe congenital Factor XI (FXI) deficiency (&lt;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\u20137.5 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.<\/jats:p>","DOI":"10.3390\/jcdd9040118","type":"journal-article","created":{"date-parts":[[2022,4,18]],"date-time":"2022-04-18T04:21:28Z","timestamp":1650255688000},"page":"118","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More"],"prefix":"10.3390","volume":"9","author":[{"given":"Toshinobu","family":"Kazui","sequence":"first","affiliation":[{"name":"Department of Surgery, The University of Arizona College of Medicine, Tucson, AZ 85724, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8072-9883","authenticated-orcid":false,"given":"Vance G.","family":"Nielsen","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, The University of Arizona College of Medicine, Tucson, AZ 85724, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Spencer D.","family":"Audie","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, The University of Arizona College of Medicine, Tucson, AZ 85724, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajagopalan M.","family":"Venkataramani","sequence":"additional","affiliation":[{"name":"Perfusion Department, Banner University Medical Center, Tucson, AZ 85719, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John T.","family":"Bryant","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, Veterans Administration Hospital, Tucson, AZ 85723, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristin","family":"Swenson","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, The University of Arizona College of Medicine, Tucson, AZ 85724, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul M.","family":"Ford","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, The University of Arizona College of Medicine, Tucson, AZ 85724, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1111\/jth.15195","article-title":"Managing the coagulopathy associated with cardiopulmonary bypass","volume":"19","author":"Bartoszko","year":"2021","journal-title":"J. Thromb. Haemost."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1157","DOI":"10.1016\/j.hoc.2021.07.012","article-title":"Factor XI Deficiency","volume":"35","author":"Lewandowska","year":"2021","journal-title":"Hematol. Oncol. Clin. N. Am."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/S0003-4975(10)62296-5","article-title":"Coronary artery bypass in hereditary factor XI deficiency","volume":"38","author":"Brunken","year":"1984","journal-title":"Ann. Thorac. Surg."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/S0888-6296(89)92882-2","article-title":"Management of factor XI deficiency in coronary artery bypass graft surgery by plasmapheresis and exchange transfusion","volume":"3","author":"Ingram","year":"1989","journal-title":"J. Cardiothorac. Anesth."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/1010-7940(90)90190-B","article-title":"The use of fresh blood to control severe haemorrhage associated with massive blood transfusion after cardiopulmonary bypass","volume":"4","author":"Macfie","year":"1990","journal-title":"Eur. J. Cardiothorac. Surg."},{"key":"ref_6","first-page":"261","article-title":"Anticoagulation during CABG in factor XI deficiency, a case report","volume":"71","author":"Nawabi","year":"1994","journal-title":"Thromb. Haemost."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1046\/j.1365-2516.2000.00385.x","article-title":"Management of factor XI inhibitor for cardiac intervention: Successful treatment with immunosuppressive therapy and plasma exchange","volume":"6","author":"Teruya","year":"2000","journal-title":"Haemophilia"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1007\/s00246-007-9081-3","article-title":"Successful use of recombinant factor VIIa (NovoSeven) during cardiac surgery in a pediatric patient with congenital factor XI deficiency","volume":"29","author":"Avci","year":"2008","journal-title":"Pediatr. Cardiol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1007\/s11748-013-0271-z","article-title":"Aortic valve replacement in an elderly patient with factor XI deficiency","volume":"62","author":"Watanabe","year":"2014","journal-title":"Gen. Thorac. Cardiovasc. Surg."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1177\/0218492316644075","article-title":"Factor XI deficiency and aortic valve replacement: Perioperative management","volume":"25","author":"Petroulaki","year":"2017","journal-title":"Asian Cardiovasc. Thorac. Ann."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e512","DOI":"10.1111\/hae.13311","article-title":"Management of severe factor XI deficiency in cardiac surgery: A case report and review of the literature","volume":"23","author":"Gay","year":"2017","journal-title":"Haemophilia"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1111\/jocs.13526","article-title":"Perioperative management of a redo aortic root replacement in a patient with severe factor XI deficiency","volume":"33","author":"Fitzsimons","year":"2018","journal-title":"J. Card Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1111\/jocs.14581","article-title":"Perioperative anesthetic management in a patient with factor XI deficiency undergoing coronary artery bypass graft surgery","volume":"35","author":"Ince","year":"2020","journal-title":"J. Card Surg."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2315","DOI":"10.1016\/j.athoracsur.2006.06.072","article-title":"Thrombin during cardiopulmonary bypass","volume":"82","author":"Edmunds","year":"2006","journal-title":"Ann. Thorac. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1213\/00000539-200004000-00008","article-title":"Anticoagulation for cardiac surgery in patients receiving preoperative heparin: Use of the high-dose thrombin time","volume":"90","author":"Manspeizer","year":"2000","journal-title":"Anesth. Analg."},{"key":"ref_16","first-page":"e349","article-title":"FXI concentrate use and risk of thrombosis","volume":"20","author":"Goudemand","year":"2014","journal-title":"Haemophilia"}],"container-title":["Journal of Cardiovascular Development and Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2308-3425\/9\/4\/118\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:54:53Z","timestamp":1760136893000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2308-3425\/9\/4\/118"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,15]]},"references-count":16,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2022,4]]}},"alternative-id":["jcdd9040118"],"URL":"https:\/\/doi.org\/10.3390\/jcdd9040118","relation":{},"ISSN":["2308-3425"],"issn-type":[{"value":"2308-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,15]]}}}