Faculty Collaboration Database

Liver disease in the patient with Fontan circulation. Congenit Heart Dis 2011;6(3):190-201

Date

03/30/2011

Pubmed ID

21443554

DOI

10.1111/j.1747-0803.2011.00504.x

Scopus ID

2-s2.0-79956263980 (requires institutional sign-in at Scopus site)   125 Citations

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.

Author List

Wu FM, Ukomadu C, Odze RD, Valente AM, Mayer JE Jr, Earing MG

MESH terms used to index this publication - Major topics in bold

Fontan Procedure
Heart Defects, Congenital
Hemodynamics
Humans
Liver Circulation
Liver Diseases
Prognosis
Pulmonary Circulation
Risk Assessment
Risk Factors