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Fontan-Associated Liver Disease: A Review

Fontan-associated liver disease is a hepatic disorder arising from hemodynamic changes and systemic venous congestion following Fontan surgery. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is not well established, over time many Fontan patients develop portal hypertension-related complications such as ascites, variceal hemorrhage or encephalopathy. Fontan survivors also show an increased risk of hepatocellular carcinoma. Early diagnosis of advanced liver disease is mandatory for the prevention and treatment of complications such as hepatocellular carcinoma, esophageal varices and malnutrition. This review updates current knowledge of the pathophysiology and management of Fontan-associated liver disease including new diagnostic methods and treatments.Fontan-associated liver disease is a hepatic disorder arising from hemodynamic changes and systemic venous congestion following Fontan surgery. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is not well established, over time many Fontan patients develop portal hypertension-related complications such as ascites, variceal hemorrhage or encephalopathy. Fontan survivors also show an increased risk of hepatocellular carcinoma. Early diagnosis of advanced liver disease is mandatory for the prevention and treatment of complications such as hepatocellular carcinoma, esophageal varices and malnutrition. This review updates current knowledge of the pathophysiology and management of Fontan-associated liver disease including new diagnostic methods and treatments.

Fontan-associated Liver Disease

Fontan-associated liver disease refers to the disturbance in the liver secondary to hemodynamic changes and systemic venous congestion following Fontan surgery. Although the natural history of this disease has not yet been established, patients with more advanced liver injury develop the complications of portal hypertension, such as ascites, variceal haemorrhage, or encephalopathy. Moreover, patients with Fontan surgery may have an increased risk of hepatocellular carcinoma. Periodic liver monitoring is essential to prevent this disease and provide early treatment of liver complications.

Incidence, predictive factors and impacts of acute kidney injury in cirrhotic patients hospitalized for cellulitis

Cellulitis is a common infection in patients with cirrhosis but its impact on progression of liver disease has been hardly addressed. This study examines the incidence of acute kidney injury (AKI), predictive factors and its impacts on mortality in cirrhotic patients hospitalized for cellulitis.

Acute variceal bleeding in patients on primary prophylaxis with nonselective beta‐blockers: A poor prognosis factor?

We read with interest the article by Ribeiro de Souza et al. addressing the impact of nonselective beta-blockers (NSBBs) on short-term prognosis in patients with cirrhosis admitted for their first episode of acute variceal bleeding (AVB). The authors adjusted their series of 49 patients on primary prophylaxis with NSBBs and 93 not on NSBBs for propensity and Model for End-Stage Liver Disease score. No influence of NSBBs used for primary prophylaxis on short-term prognosis (i.e., 5-day rebleeding and 6week mortality) was detected in patients with AVB. The message of Ribeiro de Souza et al.’s study is timely as recent publications have raised concern about the use of NSBBs for primary and secondary prophylaxis in specific clinical situations (i.e., refractory ascites). Our prospective data (January 2010-December 2015) are in line with the results of Ribeiro de Souza et al. We analyzed 106 patients with a first episode of AVB, 30 on primary prophylaxis with NSBBs (28.3%, study group) and 76 not taking NSBBs (71.7%, control group). Participants were well matched (for age, sex, etiology, previous ascites or hepatic encephalopathy, hepatocellular carcinoma, Child, and Model for End-Stage Liver Disease on admission), except for a lower heart rate in the study group (77 versus 98 bpm, P < 0.001). Rebleeding occurred in 17 of the 106 patients (16%), 2 in the study group (6.6%) and 15 in the control group (19.7%) (P 5 0.1). The odds ratio of NSBBs for bleeding was 0.3 (95% confidence interval 0.2-2.5, P 5 0.5). Fourteen of the 106 (13%) patients died during admission, 3 in the study group (10%) and 11 in the control group (14.5%) (P 5 0.5). Finally, 3 patients in the NSBB group (10%) and 15 in the control group (19.7%) died before 6 weeks (P 5 0.2). Adjusted odds ratios for in-hospital and 6-week mortality were not significantly different between the two groups: 0.5 (95% confidence interval 0.2-2.5) and 1.44 (95% confidence interval 0.1-1.7), respectively. These results reinforce the concept that variceal bleeding prophylaxis with NSBBs does not negatively affect the short-term prognosis of an AVB episode. NSBBs are the cornerstone of primary and secondary prophylaxis of variceal bleeding, and treatment with NSBBs should not be a concern if AVB arises.