Rotational thromboelastometry (ROTEM) is a viscoelastic method, which provides a graphical and numerical representation of induced hemostasis in whole blood samples. Its ability to quickly assess the state of hemostasis is used in the management of bleeding from a variety of causes. The separate activation of particular parts of hemocoagulation in INTEM, EXTEM, and FIBTEM tests allows for a more comprehensive and faster evaluation of the missing component of hemostasis followed by targeted therapy. One of the most common cause of coagulopathy is trauma-induced coagulopathy. Fibrinogen replacement therapy by ROTEM allows for the use of a standard dosage of fibrinogen, which has been shown to be successful in preventing dilutional coagulopathy following colloid and crystalloid replacement and excessive amount of allogeneic blood transfusions. The best reflection of fibrinogen activity is observed in the FIBTEM assay, where fibrinogen replacement therapy is recommended at an MCF (maximum clot firmness) of FIBTEM < 10 mm and FIBTEM A10 < 7 mm. ROTEM also plays an important role in the diagnostic and management of inherited fibrinogen disorders. These can be manifested by bleeding complications, where changes in the MCF parameter are the most useful tool for assessing the effectiveness of fibrinogen replacement therapy. ROTEM-guided bleeding management algorithms effectively reduce the number of transfusions, healthcare costs, and complications, leading to the improvement of patient safety and overall health.

Congenital fibrinogen disorders are diseases associated with a bleeding tendency; however, there are also reports of thrombotic events. Fibrinogen plays a role in the pathogenesis of thrombosis due to altered plasma concentrations or modifications to fibrinogen’s structural properties, which affect clot permeability, resistance to lysis, and its stiffness. Several distinct types of genetic change and pathogenetic mechanism have been described in patients with bleeding and a thrombotic phenotype, including mutations affecting synthesis or processing in three fibrinogen genes. In this paper, we focused on familial hypofibrinogenemia, a rare inherited quantitative fibrinogen disorder characterized by decreased fibrinogen levels with a high phenotypic heterogeneity. To begin, we briefly review the basic information regarding fibrinogen’s structure, its function, and the clinical consequences of low fibrinogen levels. Thereafter, we introduce 15 case reports with various gene mutations derived from the fibrinogen mutation database GFHT (French Study Group on Hemostasis and Thrombosis), which are associated with congenital hypofibrinogenemia with both bleeding and thrombosis. Predicting clinical presentations based on genotype data is difficult. Genotype–phenotype correlations would be of help to better understand the pathologic properties of this rare disease and to provide a valuable tool for the identification of patients who are not only at risk of bleeding, but also at risk of a thrombotic event.

Background: Perioperative assessment of hemostasis remains challenging, particularly in non-cardiac surgery, where the incidence of clinically significant bleeding is relatively low. Viscoelastic testing enables real-time evaluation of whole-blood coagulation, but its perioperative dynamics and agreement with conventional assays require further characterization. Methods: In this prospective single-center observational study, 53 patients undergoing major non-cardiac surgery were included in the final analysis. Hemostatic parameters were assessed preoperatively, intraoperatively at two-hour intervals, and 24 h postoperatively using ROTEM (EXTEM, INTEM, and FIBTEM) and conventional coagulation assays. Longitudinal changes and correlations between fibrinogen concentration according to Clauss and ROTEM-derived parameters were analyzed. Results: Fifty-three patients undergoing major hepatopancreatobiliary surgery completed longitudinal perioperative monitoring. Pancreaticoduodenectomy and liver resection accounted for 60.7% and 39.3% of procedures, respectively. Serial perioperative ROTEM and laboratory assessments demonstrated time-dependent changes in coagulation parameters, including decreased intraoperative fibrinogen and antithrombin III levels and shortening of INTEM clotting time. Clinically significant bleeding was infrequent, with severe intraoperative bleeding observed in 7.4% of patients. Strong correlations were confirmed between fibrinogen concentration according to Clauss and FIBTEM-derived parameters, particularly FIBTEM MCF (r = 0.811, p < 0.001), whereas the correlation with FIBTEM A10 was moderate (r = 0.574, p < 0.001). No significant association was observed between preoperative fibrinogen concentration and perioperative blood loss. Conclusions: This study provides a longitudinal characterization of perioperative coagulation dynamics and demonstrates strong agreement between fibrinogen concentration according to Clauss and ROTEM-derived parameters. The findings are primarily descriptive and highlight the methodological consistency between viscoelastic and conventional fibrinogen assessment. Further studies are required to determine their clinical relevance in perioperative management. These findings suggest that ROTEM-derived parameters may provide a rapid functional estimate of fibrinogen status in the perioperative setting, potentially supporting clinical assessment, although their role in guiding management requires further validation.