Coagulation factor deficiencies may predispose patients with functionally univentricular hearts to bleeding tendencies preoperatively and perioperatively. Postoperatively, this is altered to a prothrombotic predisposition in these patients. Early and late thromboembolic events secondary to a prothrombotic state following univentricular repair contribute to significant morbidity after establishment of the Fontan circulation. Anticoagulation following univentricular repair is advocated but its benefits are controversial.
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