Persistent fibrinogen deficiency can lead to bleeding complications and an increased risk in mortality. Early identification and treatment of fibrinogen deficiency could therefore save lives.
Correct perioperative management of fibrinogen loss experienced during heart surgery and postpartum haemorrhage is key to treatment success.
Fibrinogen role in haemostasis
Fibrinogen (Factor I) has many functions in haemostasis, a process that stops bleeding from damaged tissue. Such functions include coagulation (the creation of blood clots), fibrinolysis (the breaking down of blood clots), wound healing, inflammation and cell–cell interactions.
Congenital fibrinogen deficiency
Low fibrinogen levels and quality impacts negatively on blood clot formation and can lead to excessive bleeding and death if left untreated. Fibrinogen deficiencies can either be inherited (congenital fibrinogen deficiency) or acquired (acquired fibrinogen deficiency). Congenital fibrinogen deficiency manifests from no detectable fibrinogen (such as for congenital afibrinogenaemia) to some detectable fibrinogen (such as for congenital hypofibrinogenaemia) (Figure 5).
It is usually the result of a heterozygous or homozygous mutation in one of the three fibrinogen genes (FGA, FGB and FGG) that are located on the long arm of chromosome 4 (4q31.3). Key symptoms include uncontrollable bleeding and thrombolytic complications. The onset of bleeding can be spontaneous, or as a result of both minor and major traumas. Bleeding phenotypes can range from being asymptomatic to severe and is partially dependent on both the level and quality of fibrinogen available (Peyvandi et al., 2012). There are two types of congenital deficiency:
Major bleeding during cardiac surgery is an outcome that warrants the use of blood transfusions and reinterventions, both of which are risk factors for mortality (Moulton et al., 1996; Makar et al., 2010; Vivacqua et al., 2011). Acute coagulation defects are a common complication in cardiac surgery and can arise as a result of tissue injury and hypotension, with haemodilution, hyperfibrinolysis, and inflammatory responses all helping to sustain bleeding (Levy et al., 2005; Karkouti et al., 2010; Makar et al., 2010; Chee et al., 2016).
An added complication is that this patient group are likely to have comorbidities and ongoing anticoagulant treatment for the prevention of heart disease, or anticoagulants administered during surgery to prevent clot formation (such as heparin) (Scrutinio & Giannuzzi, 2008).
Postpartum haemorrhage (PPH) is a bleeding complication that accounts for approximately one in four maternal deaths, making it the most common cause of maternal death worldwide (Kassebaum et al., 2014). The incidence rate in developed countries has increased in recent years, related primarily to an increase in the use of oxytocin for progressing labour (Belghiti et al., 2011).
PPH is usually the result of uterine atony, but may be due to uterine rupture, abnormal placentation, placental abruption, genital tract trauma and coagulation defects (Abdul-Kadir et al., 2014). Comorbidities such as foetal death can also impact on PPH severity.
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