Background: Thromboembolic events are a well-known risk for Fontan patients and often lead to morbidity and mortality in cyanotic patients and patients with a single ventricle. Coagulopathy and thrombophilia, in addition to disturbed Fontan blood flow and endothelial injury, are major contributors to thromboembolic complications. However, there is currently no consensus regarding the optimal medication to prevent or treat these events. Identification of coagulation disorders is therefore crucial for selecting appropriate management strategies and evaluating long-term outcomes in these patients.
Case presentation: We present the case of a 35-year-old male who underwent the Fontan procedure with a total cavopulmonary modification during childhood due to tricuspid atresia. He was admitted with complaints of headaches and visual disturbances, but no clear cardiovascular cause was identified. Standard coagulation test parameters were normal, but the thrombodynamics test indicated severe hypercoagulation and spontaneous clot formation. Anticoagulation therapy was initiated, and a subsequent thrombodynamics assay showed normalization of the coagulation parameters. The patient remained asymptomatic during the six-month follow-up period.
Conclusions: The thrombodynamics test is a valuable tool for the diagnosis of coagulation disorders, as it can assess coagulation parameters and clot growth in vitro. This method can also aid in the optimization of antithrombotic therapy. The presented clinical case highlights the potential use of the thrombodynamics test in Fontan patients to diagnose coagulation disorders and improve long-term outcomes.
Keywords: ACHD; Case report; Fontan; Hemostasis; Thromboembolic events.
© 2023. The Author(s).