LIVER, PANCREAS AND BILIARY TRACT: Clinical ReviewLiver Disease After the Fontan Procedure What the Hepatologist Needs to KnowShah, Hiral MD*; Kuehl, Karen MD†; Sherker, Averell H. MD, FRCP(C)*Author Information *Division of Gastroenterology and Hepatology, Department of Medicine, Washington Hospital Center †Adult Congenital Heart Diseases, Children's National Medical Center, Washington, DC Funding: None Reprints: Hiral Shah, MD, Washington Hospital Center, Division of Gastroenterology and Hepatology, 110 Irving Street, North West, Washington, DC 20010 (e-mail: [email protected]). Conflict of Interest: None Journal of Clinical Gastroenterology: July 2010 - Volume 44 - Issue 6 - p 428-431 doi: 10.1097/MCG.0b013e3181d476fc Buy Metrics Abstract Fifty years ago, only a quarter of infants born with congenital heart disease (CHD) survived beyond the first year of life. It has been estimated that 80% to 85% of patients can expect to reach adulthood. Today, there are close to 1 million adults living with CHD in the United States alone. Since its introduction, the Fontan operation has become established as the dominant surgical repair in patients with univentricular heart physiology. Long-term morbidity associated with this procedure is being more commonly appreciated and liver complications have become more frequently apparent. Hepatologist awareness and familiarity in managing this population alongside the CHD specialist is important for the care of these patients. This dual perspective can provide comprehensive integrated care. © 2010 Lippincott Williams & Wilkins, Inc.