Hepatic dysfunction following the Fontan procedure.
OBJECTIVE
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.
Narkewicz
Michael R
MR
Department of Pediatrics, University of Colorado School of Medicine and The Children's Hospital, Denver, 80218, USA. narkewicz.michael@tchden.org
Sondheimer
Henry M
HM
Ziegler
James W
JW
Otanni
Yvonne
Y
Lorts
Angela
A
Shaffer
Elizabeth M
EM
Horgan
J Gerard
JG
Sokol
Ronald J
RJ
D000293:Adolescent; D001780:Blood Coagulation Tests; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D005690:Galactose / Q000493:pharmacokinetics*; D006321:Heart / Q000502:physiology; D006330:Heart Defects, Congenital / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D008107:Liver Diseases / Q000097:blood / Q000209:etiology*; D008111:Liver Function Tests; D008297:Male; D011517:Prothrombin Time; D014463:Ultrasonography
D005690:Galactose