Liver Disease in the Patient with Fontan Circulation
- 28 March 2011
- journal article
- review article
- Published by Computers, Materials and Continua (Tech Science Press) in Congenital Heart Disease
- Vol. 6 (3), 190-201
- https://doi.org/10.1111/j.1747-0803.2011.00504.x
Abstract
The Fontan procedure has undergone many modifications since first being performed on a patient with tricuspid valve atresia in 1968. It is now the procedure of choice for individuals born with single-ventricle physiology or for those in whom a biventricular repair is not feasible. Forty years of experience with the Fontan procedure have gradually revealed the shortfalls of such a circulatory arrangement. Sequelae related to the underlying congenital anomaly or to the altered physiology of passive, nonpulsatile flow through the pulmonary arterial bed can result in failure of the Fontan circulation over time. Liver abnormalities including abnormalities in the clotting cascade have been well documented in Fontan patients. The clinical significance of these findings, however, has remained poorly understood. As Fontan survivors have increased in age and number, we have begun to better recognize subclinical hepatic dysfunction and the contribution of liver disease to adverse outcomes in this population. The purpose of this review is to summarize the existing data pertaining to liver disease in the Fontan population and to identify some questions that have yet to be answered.Keywords
This publication has 70 references indexed in Scilit:
- Liver function abnormalities and outcome in patients with chronic heart failure: data from the Candesartan in Heart Failure: Assessment of Reduction in Mortality and Morbidity (CHARM) programEuropean Journal of Heart Failure, 2009
- Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological studyJournal of Clinical Pathology, 2007
- Liver fibrosis: a balance of ACEs?Clinical Science, 2007
- Hepatic changes in the failing Fontan circulationHeart, 2007
- Systemic Oxygen Saturation and Coagulation Factor Abnormalities Before and After the Fontan ProcedureThe American Journal of Cardiology, 2005
- Juvenile hepatocellular carcinoma with congestive liver cirrhosisThe Esophagus, 2005
- Decreasing fibrogenesis: an immunohistochemical study of paired liver biopsies following lamivudine therapy for chronic hepatitis BJournal of Hepatology, 2001
- Prevalence and Clinical Outcome of Hepatitis C Infection in Children Who Underwent Cardiac Surgery before the Implementation of Blood-Donor ScreeningThe New England Journal of Medicine, 1999
- The Prevalence of Hepatitis C Virus Infection in the United States, 1988 through 1994The New England Journal of Medicine, 1999
- Functional deterioration of the liver by elevated inferior vena cava pressure: A proposed upper safety limit of pressure for maintaining liver viability in dogsIntensive Care Medicine, 1994