Hepatic Abnormalities Are Present Before and Early After the Fontan Operation.

BACKGROUND

Progressive hepatic fibrosis is common after the Fontan operation, but little is known about its onset. We sought to determine whether hepatic abnormalities before the Fontan operation, and whether further abnormalities can be identified soon afterward.

METHODS

Patients undergoing the Fontan operation at our institution were prospectively enrolled and underwent hepatic ultrasound with Doppler and serum testing immediately before and 3 to 6 months after the operation.

RESULTS

Thirty patients were enrolled at a median age of 3.1 years (range, 2.2 to 8.1 years). Extracardiac Fontan was performed in 67%. Three patients (10%) had abnormal hepatic echotexture before the operation. At the post-Fontan study, mean liver length increased (9.9 versus 10.9 cm; p < 0.0001), and mean hepatic artery end-diastolic velocity decreased (18.8 versus 14.5 cm/s; p = 0.03). One patient showed new, abnormal hepatic echotexture after surgery. Mean aspartate aminotransferase (56.7 versus 60.7 U/L; p = 0.04), alanine transaminase (18.9 versus 33.9 U/L; p = 0.0002), and γ-glutamyl transferase (18.7 versus 46.1 U/L; p = 0.002) increased at the post-Fontan assessment compared with the preoperative values. By linear regression, hospital length of stay and duration with chest tube after Fontan operation were both significantly associated with an increase in γ-glutamyl transferase (p < 0.001 for both) and alanine transaminase (p = 0.008, p = 0.048).

CONCLUSIONS

In this cohort, hepatic abnormalities were observed in some patients before the Fontan operation and new abnormalities developed soon afterward. These findings suggest that liver insult may occur before or soon after the Fontan operation.