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There are approximately one million adult patients with congenital heart disease (CHD) in the United States and the number is increasing. Hepatic complications are common and may occur secondary to persistent chronic passive venous congestion or decreased cardiac output due to the underlying cardiac disease, or as a result of palliative cardiac surgery; transfusion or drug related hepatitis may also occur. The unique physiology of Fontan circulation is particularly prone to development of hepatic complications and is in part related to the duration of the Fontan procedure. Liver biochemical test abnormalities may be related to cardiac failure, due to intrinsic liver disease, secondary to palliative interventions, or drug-related. Complications of portal hypertension and rarely, hepatocellular carcinoma may also occur. Abnormalities such as hypervascular nodules are often seen; in the presence of cirrhosis surveillance for hepatocellular carcinoma is necessary. Judicious perioperative support is required when cardiac surgery is performed in patients with advanced hepatic disease. Traditional models for liver disease staging may not fully capture the severity of disease in patients with CHD. The effectiveness or safety of isolated liver transplantation in patients with significant CHD is limited in adults; combined heart-liver transplantation may be required in those with decompensated liver disease or hepatocellular carcinoma, but experience is limited in the presence of significant CHD. The long term sequelae of many reparative cardiac surgical procedures are not yet fully realized; understanding the unique and diverse hepatic associations and the role for early cardiac transplantation in this population is critical. As this population continues to grow and age, consideration should be given to developing consensus guidelines for a multidisciplinary approach to optimize management of this vulnerable population. (HEPATOLOGY 2012.).
Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota.
This article was published in the following journal.
Name: Hepatology (Baltimore, Md.)
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