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The Fontan procedure (FP) has been used to separate the systemic and pulmonary circulations in patients with various forms of functionally univentricular hearts since 1971 (Fontan & Baudet, 1971). Ventricle function is attempted to be preserved under optimal conditions by this operation. The Fontan procedure is a highly effective palliative operation for infants and provides increased survival and improved quality of life. However, there are still significant problems after the completion of the FP, including heart failure, arrhythmias, thrombotic complications, congestive hepatopathy, and protein-losing enteropathy (Ghaferi & Hutchins, 2005).
In the absence of hydraulic force of the right ventricle, Fontan circulation results in a paradox of systemic venous hypertension and pulmonary artery hypotension. It has detrimental effects on infradiaphragmatic venous and splanchnic circulation. Chronic venous congestion in the liver leads to increased hepatic sinusoidal pressure that leads to hepatic fibrosis, cirrhosis, and portal venous hypertension (Asrani et al., 2012; Kiesewetter et al., 2007; Schwartz et al., 2012). Dysfunction of the ventricle after years of systemic loading induces impaired compliance, poor ventricular filling, and eventually low cardiac output (Asrani et al., 2012; Jolley, Colan, Rhodes, & DiNardo, 2015; Kiesewetter et al., 2007). Hypoxy and ischemia expedite hepatic injury undoubtedly. Hepatocellular carcinoma (HCC) might occur on this background (Asrani et al., 2012; Asrani, Warnes, & Kamath, 2013; Jolley et al., 2015). Here, we report a case with congenital heart disease and the FP, representing liver disease and cancer at an adult age. Informed consent was taken from the patient and his parents to share this case study.

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