Liver disease more prominent in Fontan surgery patients, research shows

New research from the Children’s Hospital of Philadelphia shows patients who have undergone the Fontan operation as children are at an increased risk for developing progressive liver fibrosis.

The study, published in the Journal of the American Heart Association, dives into how the circulation created by the Fontan surgery affects the onset of liver disease. The operation is a series of three staged reconstructive surgeries in children born with single-ventricle disease and re-routes blood to the lungs, which can sometimes damage many organ systems. The findings from the study underscore the importance of improving ongoing medical surveillance.

"We have known for some time that liver fibrosis is a complication of Fontan surgery, but this was an important study that demonstrated that the length of time after the Fontan operation is a significant contributor to the degree of fibrosis," said lead study author David J. Goldberg, MD, a pediatric cardiologist at the children’s hospital, in a statement.

In Goldberg’s study, investigators analyzed 67 children and adolescents treated between 2009 and 2014 in the hospital’s Single Ventricle Survivorship Program. Most patients were evaluated in the program 10 to 15 years after they underwent their first Fontan operation.

All 67 children had signs of some liver fibrosis, though most of their conditions were not extremely dangerous.

"Our finding that liver fibrosis is universal after the Fontan operation reinforces the need for ongoing surveillance of the liver in this patient population," Goldberg said. "However, it is important to find a less invasive method than liver biopsy to measure liver fibrosis, as well as to investigate possible medications that may reduce the rate of fibrosis. As we work to develop clinical guidelines for liver surveillance, we can better provide care tailored to our individual patients."