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Background: Transient elastography (TE) offers a noninvasive correlate with the degree of hepatic fibrosis. However, factors other than fibrosis affect liver stiffness. We sought to determine whether hepatic congestion related to hemodynamics in Fontan circulation influences liver stiffness measurement (LSM) assessed by TE., Methods: We studied 45 subjects with Fontan circulation undergoing cardiac catheterization with or without simultaneous liver biopsy. Subjects underwent TE within 5 days before catheterization. Clinical history, hemodynamic and biopsy data, and hepatic biomarkers were collected. Five subjects who had previously undergone liver biopsy and TE were also included., Results: Median age was 13.1 years (range 2.4-57.8); median time since Fontan was 9.9 years (range 0.1-32.5). No subject had known hepatitis C. Mean LSM for the entire cohort was 21.4 ± 10.8 kPa. Univariate regression analysis using LSM as a continuous outcome variable shows significant correlations with age (R = 0.35, P = .01), time since Fontan (R = 0.41, P = .003), Fontan pressure (R = 0.31, P = .04), cardiac index (R = 0.33, P = .03), pulmonary vascular resistance (R = 0.34, P = .03), systemic arterial oxygen saturation (R = 0.31, P = .04), and platelet count (R = 0.29, P = .05). On multiple regression analysis, Fontan pressure (β = 0.901, P = .03) and cardiac index (β = 2.703, P = .02) were significant predictors of LSM with overall model R(2) = 0.206. Univariate analysis shows LSM to be associated with more severe centrilobular fibrosis (P = .05)., Conclusions: Higher LSM is associated with unfavorable Fontan hemodynamics and advanced centrilobular hepatic fibrosis. TE may be a useful tool for identifying Fontan patients who warrant invasive testing., (© 2014 Wiley Periodicals, Inc.)

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., (© 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.)