Hepatic pathology may develop before the Fontan operation in children with functional single ventricle: an autopsy...

Liver fibrosis has emerged as an important long-term complication of the Fontan operation. We aimed to describe liver histology at autopsy in patients who had undergone the Fontan operation and to determine whether patient variables are associated with the degree of fibrosis.

A review was performed of all patients with a history of the Fontan operation who died and underwent autopsy at our institution from 1980 to 2009. Autopsy liver slides were evaluated independently by 2 pathologists.

Twenty-two patients were studied. The median interval between Fontan and death was 20 days (range, 1 day-17.5 years). Portal fibrosis was observed in 20 (91%) patients and sinusoidal fibrosis was observed in 17 (77%) patients. Using simple linear regression, time from the Fontan operation was significantly associated with the degree of portal fibrosis on Ishak (P = .03) and modified Scheuer fibrosis (P = .02) scales. Significant portal fibrosis was observed in 8 (57%) of the 14 patients who died 30 days or less after the Fontan operation. In these 14 patients, severity of portal fibrosis was associated with length of hospitalization after pre-Fontan cardiac operations (P = .03) and pre-Fontan mean right atrial pressure (P = .04).

At autopsy, hepatic fibrosis was commonly observed in patients who had undergone the Fontan operation. Portal fibrosis has been previously unrecognized in this population. Significant portal fibrosis occurred in most who died soon after the Fontan procedure and was associated with pre-Fontan morbidity. Hepatic disease in the single-ventricle population is multifactorial and may begin before the Fontan operation.