Liver and cardiac function in the long term after Fontan operation.

Abstract

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.

• Authors: Sonia Camposilvan, Ornella Milanesi, Giovanni Stellin, Andrea Pettenazzo, Lucia Zancan, Lorenzo D'Antiga
• Journal: The Annals of thoracic surgery (Ann Thorac Surg) Vol. 86 Issue 1 Pg. 177-82 (Jul 2008) ISSN: 1552-6259 [Electronic] Netherlands
• PMID: 18573420 (Publication Type: Journal Article)

Topics

• Adolescent
• Adult
• Age Distribution
• Child
• Child, Preschool
• Cohort Studies
• Evaluation Studies as Topic
• Female
• Follow-Up Studies
• Fontan Procedure (adverse effects, methods)
• Heart Defects, Congenital (diagnosis, surgery)
• Heart Function Tests
• Humans
• Incidence
• Liver Function Tests
• Male
• Postoperative Complications (diagnosis, epidemiology)
• Probability
• Prognosis
• Quality of Life
• Risk Assessment
• Sex Distribution
• Time Factors