Hepatic Dysfunction Following the Fontan Procedure

Narkewicz, Michael R.*; Sondheimer, Henry M.†; Ziegler, James W.†; Otanni, Yvonne†; Lorts, Angela†; Shaffer, Elizabeth M.†; Horgan, J. Gerard‡; Sokol, Ronald J.*

Journal of Pediatric Gastroenterology & Nutrition:
Original Articles: Hepatology and Nutrition

Objectives: The Fontan procedure offers a palliation for the hemodynamic derangements associated with congenital heart lesions characterized by a single functional ventricle, but it causes a chronically elevated systemic venous pressure that may result in hepatic congestion. The objective of this study was to characterize hepatic function and its relationship to cardiac function in children who had undergone the Fontan procedure.

Methods: In a cross-sectional study of 11 children aged 38 months to 216 months (mean, 149 months), the authors evaluated indices of cardiac and hepatic function, including galactose clearance, Doppler hepatic ultrasonography, synthetic function, and markers of liver injury, at 9 months to 176 months (mean, 100 months) after children had undergone the Fontan procedure.

Results: The most common biochemical abnormality of hepatic function was a prolongation of the prothrombin time and a low factor V level. There was a trend toward progressive abnormality in prothrombin time with increasing interval since the Fontan procedure. Galactose elimination half-life and galactose elimination capacity were inversely correlated with the time after Fontan (R2 = 0.65, P = 0.004). There was no relationship between cardiac functional measurements and liver function.

Conclusions: Prothrombin time and galactose elimination half-life are abnormal in children who have undergone the Fontan procedure and may be useful markers of hepatic function in the longitudinal assessment of these patients.

Author Information

Sections of *Pediatric Gastroenterology, Hepatology and Nutrition and the Pediatric Liver Center, and †Pediatric Cardiology, Department of Pediatrics, University of Colorado School of Medicine and The Children's Hospital, and the ‡Department of Radiology, Presbyterian St. Luke's Hospital, Denver, Colorado, U.S.A.

Received April 22, 2002; accepted October 28, 2002.

Supported by grant 5 M01 RR00069, General Clinical Research Centers Program, National Center for Research Resources, NIH.

Presented in part at the North American Society for Pediatric Gastroenterology and Nutrition meeting in 1996 and published in abstract form (J Pediatr Gastroenterol Nutr 1996;23:351).

Address correspondence and reprint requests to Dr. Narkewicz, Children's Hospital, B290, 1056 East 19th Ave., Denver, CO 80218, U.S.A. (e-mail: narkewicz.michael@tchden.org). Dr. Ziegler's current address is Department of Pediatrics, 593 Eddy St., Providence, RI 02903, U.S.A.

© 2003 Lippincott Williams & Wilkins, Inc.