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.
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Electronic address: matthew.schwartz@orlandohealth.com.
Matthew C
MC
Schwartz
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Andrew C
AC
Glatz
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Kaitlyn
K
Daniels
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
David J
DJ
Goldberg
Division of Gastroenterology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Elizabeth
E
Rand
Division of Radiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Monica S
MS
Epelman
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Meryl S
MS
Cohen
D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008103:Liver Cirrhosis / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D011183:Postoperative Complications; D011446:Prospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D018608:Ultrasonography, Doppler