Digestive Health CenterGastroenterology, Hepatology & Nutrition

Congenital Heart Disease (Fontan) Liver Clinic

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The UCSF Congenital Heart Disease (Fontan) Liver Clinic is the only program in Northern California that provides comprehensive screening and care for liver disease in children and adults with congenital heart disease caused by a single ventricle heart defect. People with this defect were born with one functional heart ventricle instead of two.

At our clinic, we conduct liver disease screening for all UCSF patients who have undergone the Fontan procedure. This lifesaving surgery for people with single ventricle defects – usually done in early childhood – changes the way blood flows through the heart, lungs and liver.

As kids grow into adolescence and adulthood, their changed circulation can affect the liver and put them at risk for developing chronic liver disease, such as fibrosis or cirrhosis. Some people with other types of congenital heart disease are also at risk for chronic liver disease and come to our clinic for care.

We collaborate with specialists in cardiology, gastroenterology and hepatology, and pediatric radiology to help manage the effects of congenital heart disease on the liver and optimize your care.

Initial evaluation for liver dysfunction and disease

Our evaluation includes:

  • A visit with a hepatologist (a liver disease specialist) with expertise in Fontan-associated liver problems
  • A specialized liver ultrasound to check for the buildup of scar tissue that occurs with fibrosis
  • Lab screening for liver dysfunction and liver disease
  • Screening for significant liver damage and portal hypertension (high blood pressure in the vein that brings blood to the liver)
  • Counseling on how to avoid other common causes of liver damage
  • Educational resources and recommendations from our team

The hepatologist will review the evaluation results and meet with you to discuss further testing, recommended treatment and preventive care to minimize the risk of future liver problems. A UCSF cardiologist is available to consult with the hepatologist and review your or your child's most recent cardiac evaluation.

Follow-up care

Based on the initial evaluation, our liver specialists can provide individualized follow-up care.

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    What to Bring

    • Photo I.D.
    • Health insurance card
    • Insurance authorization, if required
    • Doctor's referral, if required
    • Recent test results related to your child's condition
    • List of medications, including dosages, plus any your child is allergic to
    • List of questions you may have
    • Device or paper for taking notes

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