Identifying predictors of hepatic disease in patients after the Fontan operation: a postmortem analysis.
OBJECTIVE
We characterized the histologic liver changes that occur after the Fontan operation and identified the factors associated with worsening hepatic disease.


METHODS
From 1979 to 2009, 33 patients who died after the Fontan operation had a postmortem analysis at our institution. The liver histologic findings were graded for sinusoidal and portal fibrosis.


RESULTS
The median interval from the Fontan operation to death was 14 days (range, 0 days to 25.6 years). The median age at death was 7.7 years (range, 1.9-52.9 years). Hepatic sinusoidal fibrosis occurred in 25 of 33 patients (76%), with significant fibrosis (grade 2 or greater) in 15 of 25 patients (60%). Portal fibrosis occurred in 17 of 33 patients (52%). The degree of sinusoidal (P < .003) and portal fibrosis (P < .002) correlated with an increased period from the Fontan operation to death. Sinusoidal fibrosis was also associated with increased age at the initial Fontan operation (P < .03). Of the patients who died early (< 35 days postoperatively) after the Fontan operation, sinusoidal fibrosis was present in 15 of the 23 patients (65%) and portal fibrosis in 7 of 23 patients (30%). Significant (grade 2 or greater) sinusoidal fibrosis was seen in 7 patients, and 4 of the 7 had heterotaxy syndrome. Significant portal fibrosis was observed in only 2 patients; both had heterotaxy syndrome.


CONCLUSIONS
The interval from the initial Fontan operation to death is associated with increased sinusoidal and portal fibrosis. In this small cohort, older age at Fontan operation was associated with sinusoidal fibrosis. Significant sinusoidal fibrosis was observed in patients who died early after the Fontan operation, with more severe disease present in patients with heterotaxy syndrome.
Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minn 55905, USA.
Jennifer A
JA
Johnson
Frank
F
Cetta
Rondell P
RP
Graham
Thomas C
TC
Smyrk
David J
DJ
Driscoll
Sabrina D
SD
Phillips
Anitha S
AS
John
D000293:Adolescent; D000328:Adult; D001344:Autopsy; D002648:Child; D002675:Child, Preschool; D005355:Fibrosis / Q000209:etiology; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology*; D008107:Liver Diseases / Q000209:etiology* / Q000473:pathology; D012189:Retrospective Studies; D055815:Young Adult