Liver and cardiac function in the long term after Fontan operation.
BACKGROUND
Patients who underwent Fontan operation have some degree of liver disease. We aimed to assess the long-term liver and cardiac function after Fontan operation.


METHODS
Patients enrolled underwent physical examination, biochemical tests (aspartate aminotransferase, alanine aminotransferase, gamma glutamyl transpeptidase, bilirubin, international normalized ratio, coagulation factor V, protein profile, fecal alpha-1-antitrypsin), echocardiogram, and liver ultrasonography. A liver disease score was adopted to compare the degree of liver involvement with hemodynamic features.


RESULTS
The study enrolled 34 patients, median age 14.7 years (range, 4.1 to 26.7), 26 with a residual left ventricle, 8 with a residual right ventricle, affected by tricuspid atresia (17), pulmonary atresia (4), hypoplastic left heart syndrome (5), double-outlet right ventricle (2), single left ventricle (2), and miscellaneous (4), with median follow-up of 11.5 years (range, 1.7 to 23.3). We found hepatomegaly in 18 of 34 (53%), splenomegaly in 3 of 33 (9%), abnormal transaminases in 10 of 33 (30%), elevated gamma GT in 19 of 31 (61%), elevated bilirubin in 10 of 31 (32%), coagulopathy in 17 of 29 (58%), and protein-losing enteropathy in 4 of 21 (19%). Median heart rate z-score was -1.72. Hepatic dysfunction was strictly correlated to low cardiac index (r(2) = 0.34, p = 0.008) and to a lesser extent to reduced heart rate (r(2) = 0.18, p = 0.07).


CONCLUSIONS
In children who underwent Fontan operation, hepatic dysfunction is correlated with low cardiac index and reduced heart rate. Maintaining or reestablishing a normal cardiac index might prevent or reduce liver disease in the long-term.
Camposilvan
Sonia
S
Department of Pediatric Hepatology, University of Padova, Padova, Italy.
Milanesi
Ornella
O
Stellin
Giovanni
G
Pettenazzo
Andrea
A
Zancan
Lucia
L
D'Antiga
Lorenzo
L
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005069:Evaluation Studies as Topic; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects* / Q000379:methods; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006334:Heart Function Tests; D006801:Humans; D015994:Incidence; D008111:Liver Function Tests; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis* / Q000453:epidemiology; D011336:Probability; D011379:Prognosis; D011788:Quality of Life; D018570:Risk Assessment; D017678:Sex Distribution; D013997:Time Factors