부산대학교 도서관

There are approximately one million adult patients with congenital heart disease (CHD) in the United States and the numberis increasing. Hepatic complications are common and may occur secondary to persistent chronic passive venous congestion ordecreased cardiac output due to the underlying cardiac disease, or as a result of palliative cardiac surgical procedures performedin infancy or childhood; transfusion or drug related hepatitis may also occur. The unique physiology of Fontan circulation is particularlyprone to development of hepatic complications and is in part related to the duration of the Fontan procedure. Liver biochemicaltest abnormalities may be related to cardiac failure, due to intrinsic liver disease, secondary to palliative interventions,or drug-related. Ascites, hemorrhage from gastro-esophageal varices, portal vein thrombosis, and rarely, hepatocellular carcinomamay also occur. Abnormalities such as hypervascular nodules are often seen; in the presence of cirrhosis surveillance forhepatocellular carcinoma is necessary. Judicious perioperative support is required when cardiac surgery is performed in patientswith advanced hepatic disease. Traditional models for liver disease staging may not fully capture the severity of disease in patientswith CHD. The effectiveness or safety of isolated liver transplantation in patients with significant CHD is limited in adults; combinedheart-liver transplantation may be required in those with decompensated liver disease or hepatocellular carcinoma, butexperience is limited in the presence of significant CHD. The long term sequelae of many reparative cardiac surgical proceduresare not yet fully realized, and understanding the unique and diverse hepatic associations and the role for early cardiac transplantationin this population is critical. As this population continues to grow and age, consideration should be given to developconsensus guidelines for a multidisciplinary approach to optimize management of this vulnerable population.