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Conflicting Hemodynamic Goals in an Adult Patient With Fontan Physiology Presenting for Resection of a Hepatocellular Carcinoma.

THE FONTAN PROCEDURE is used to treat children with congenital heart disease resulting in a single functional ventricle. Caval flow is directed to the pulmonary arteries without the aid of a subpulmonic ventricle. The absence of a right ventricle requires chronically elevated central venous pressure (CVP), which must be maintained at a sufficient level to drive pulmonary blood flow and maintain cardiac output. This pressure is transmitted to the liver, causing chronic hepatic congestion, which may be exacerbated by lower mean arterial perfusion pressures and chronic hypoxemia. These factors contribute to the increased rates of liver inflammation, gastroesophageal varices, liver nodules, and increasing reports of hepatocellular carcinoma (HCC) in these patients. In patients with Fontan circulation, a precipitous drop in preload can result in low cardiac output and hypotension. In patients requiring liver resection, elevated CVP is transmitted via the hepatic veins in the surgical bed, resulting in increased intraoperative bleeding. This leads to the practice of targeting CVP of less than 5 mmHg for major hepatectomy. These conflicting hemodynamic goals are examined in this case report.