Fontan Surgery and the Liver

Recently, more and more people have been mentioning possible liver issues after the Fontan surgery. We wanted to find out more information so we sat down with Dr. Frank Cetta, Chair, Pediatric Cardiology at Mayo Clinic to discuss the topic.

Q: There has recently been some talk about possible issues with Fontan surgery and the liver. What exactly does this mean and why are we just beginning to talk about it?

A: Within the last five to ten years, doctors have become more aware of possible issues the Fontan surgery poses for the liver. This isn’t just an issue for people with HLHS but for anyone who has had the Fontan surgery. Sometimes, what can happen is that the right side of the heart has more pressure on it which leads to increased pressure in the veins and then on the liver as well. It doesn’t happen in every case, but we are now becoming more attuned to it due to advancements in diagnostic tools.

Q: Is liver surveillance routine now after the Fontan surgery?

A: Yes, liver surveillance is now routine after the Fontan surgery. Most doctors think that by ten years out from the procedure, it is time to start testing to see how the liver is functioning. Possible liver issues are slow progressing which is why doctors begin testing anywhere from ten to twenty years after the Fontan surgery.

Q: What types of tests are available to check on the liver’s function?

A: Right now, care teams begin examining the liver about ten years out from the Fontan surgery using a combination of simple imaging, such as an ultrasound, or more complicated imaging like an MRI. We used to use blood tests to check on the liver, but we have found that liver problems show up later in the blood than they do using imaging diagnostics. We are also using a cutting-edge technology called Magnetic Resonance Elastography (MRE) that was invented here at Mayo Clinic. MRE works by using sound waves and MRI imaging to view body tissues. This advanced screening tool makes it possible to detect liver issues earlier than with previous tools.

Q: Can anything be done to prevent liver issues with the Fontan?

A: The good news is that we are aware of the possible issues nowadays, and we are finding these issues more often and earlier because we have developed better imaging than what we had previously. We are also working on better diagnostic tools. The gap right now is treatment, and the bigger gap is prevention. The solution would be to not have a Fontan, but that doesn’t help because there are many good reasons why we do the Fontan surgery.

What needs to be done to prevent liver issues is to reduce the systemic venous pressure (pressure in inferior vena cava), and reduce the pressure placed on the liver. It is easily said but not easily accomplished.

Q: What should I do if I am nervous about my child having the Fontan surgery?

A: It is certainly understandable to be nervous heading into any procedure. The most important thing to do though is to work with your child’s care team in order to find the best possible solution for your child. There are still many good reasons why the Fontan surgery is performed and why it results in positive outcomes.