UCSF’s Fontan Liver Clinic started in 2015 as a multi-disciplinary collaboration between pediatric hepatology, pediatric cardiology, the adult congenital heart disease cardiologists, and pediatric radiology. We currently run see children and adults with congenital heart disease, including post-Fontan and other single ventricle heart defects, in both Oakland (Dr. Prita Mohanty) and San Francisco (Dr. Emily Perito, Dr. Sue Rhee), within the pediatric clinic and the Adult Congenital Heart Disease program. We also provide inpatient consultation and care for children undergoing heart transplant evaluations or with other complications of liver disease related to their congenital heart disease.

Liver disease in patients with congenital heart disease

Many patients with congenital heart disease, and all patients who have had Fontan procedures for congenital heart disease, are at risk for developing liver damage over time. Liver damage may start even before a Fontan procedure in children with congenital heart abnormalities because of high pressures in the heart that cause high back pressures and slow blood flow through the liver, as well as decreased flow of oxygenated blood from the heart back into the liver.

The Fontan procedure directs blood flow from the liver directly into the pulmonary arteries instead of into the right side of the heart (because the right side of the heart is used to pump blood returning from the lungs forward to the body). This alteration in blood flow worsens chronic congestion in the liver. Patients without a Fontan who have poor right-sided heart function can also develop liver congestion. Over time, this congestion, with slow blood flow through the liver and increased blood pressures in the liver, can damage the liver and lead to the formation of fibrosis (scar tissue). Some people with congenital heart disease and/or who have had a Fontan procedure develop severe liver damage and scarring, a condition called cirrhosis.

We worry about scarring and cirrhosis in the liver because it increases the risk of liver dysfunction and liver tumors. Patients with a lot of scarring in the liver can also develop a condition called portal hypertension that leads to fluid accumulation in the belly (ascites) and increases the risk of bleeding (varices). As these patients grow into adulthood, they also commonly develop liver masses, most of which are benign (non-cancerous) but some of which can develop into liver cancers. Recent studies have shown that liver scarring and dysfunction can effect survival in patients with congenital heart disease, particularly those who have had Fontan procedures. Our screening tests in the Fontan Liver Clinic are designed to pick up any early signs of these problems so that they can be effectively treated before they cause problems.

Other liver diseases that can affect people with congenital heart disease

We also screen for common liver diseases that are not related to congenital heart disease or to the Fontan procedure. These liver diseases can worsen liver damage in post-Fontan patients. In particular, this includes: viral hepatitis, non-alcoholic fatty liver disease, alcoholic fatty liver disease, and other liver diseases as indicated by each patient’s own clinical history.

We know that the risk of significant liver problems increases with age and time since the Fontan surgery, but not enough is known about this condition to predict who will develop significant liver damage or cirrhosis, or when it will develop. However, we can pick up early stages of liver disease with physical exam, lab tests, and imaging. We recommend ongoing monitoring of liver health and for signs of liver problems in all patients with single-ventricle congenital heart diseases and/or who have undergone a Fontan procedure.

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