HLHS Cause to Cure

Hypoplastic Left Heart Syndrome Program from Mayo Clinic

Share this:
June 2, 2015

Fontan Surgery and the Liver

By Suzanne Ferguson

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.HLHS heart with label

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.

 

 

 

 

 

------------------------------------------------------------------------------------------------------------------------------------
The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of delaying or preventing heart failure for individuals affected by congenital heart defects including HLHS. The specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies.

 

Tags: CHD, Fontan, Fontan Procedure, HLHS, Mayo Clinic, Mayo Clinic, Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome

Do the same issues exist for hypoplastic right heart syndrome? My grandson has hypoplastic right and we see very little written about It.

Liked by skigirl72, Kameg

What is the percentage of people who have the Fontan and develop liver issues?

Our son is hypoplastic right heart also & is having his Fontan at the end of this year. Do the liver issues effect HRHS kids after the Fontan?

Thanks for your question. This issue is for anyone with a single ventricle following the Fontan surgery. That being said, it is still rare and develops over several years. But it is important to stay informed about all of the possible risks. Be sure to visit our blog frequently because the a lot of information on hypoplastic left heart syndrome is applicable to hypoplastic right heart syndrome as well.
HLHS Social Media Team

@leslie1776

What is the percentage of people who have the Fontan and develop liver issues?

Jump to this post

In a recent study at Mayo Clinic, presented at ISHLT, one percent of patients who had the Fontan surgery developed liver cirrhosis ten years out, six percent of patients developed liver cirrhosis twenty years out and forty-three percent developed liver cirrhosis thirty years out.

@kafennig

Do the same issues exist for hypoplastic right heart syndrome? My grandson has hypoplastic right and we see very little written about It.

Jump to this post

I agree. My daughter is HRHS. 29 years old. Always looking for anything new

@kafennig

Do the same issues exist for hypoplastic right heart syndrome? My grandson has hypoplastic right and we see very little written about It.

Jump to this post

This is wonderful to hear. How is your daughter doing? How is her quality of life? Has she not needed a transplant? We have not met any HRHS that have reached adulthood.

Kathleen: She is doing very well. She just celebrated her first wedding anniversary this past June. She works full time as a cardiac tech. We are from Philadelphia,Pa and they have a wonderful single ventricle clinic. It was not easy transitioning from pediatric cardiologist, but hers made it a bit easier since he was retiring. Her cardiologist seems to be very pleased with her condition. No liver issues and heart function is doing well. She is a bit blue but not that others really notice. Her oxygen levels are always 89-92 which is her normal. All in all, she’s living a fairly normal life. I say fairly because it’s something I think of and know she really can’t keep up certain paces, but if you just met her you wouldn’t know she had an extremely rough time in the beginning. I hope this helps you. I sure wish I had this hope when she was little. No complaints. She’s good and we are blessed.

@kameg

Kathleen: She is doing very well. She just celebrated her first wedding anniversary this past June. She works full time as a cardiac tech. We are from Philadelphia,Pa and they have a wonderful single ventricle clinic. It was not easy transitioning from pediatric cardiologist, but hers made it a bit easier since he was retiring. Her cardiologist seems to be very pleased with her condition. No liver issues and heart function is doing well. She is a bit blue but not that others really notice. Her oxygen levels are always 89-92 which is her normal. All in all, she’s living a fairly normal life. I say fairly because it’s something I think of and know she really can’t keep up certain paces, but if you just met her you wouldn’t know she had an extremely rough time in the beginning. I hope this helps you. I sure wish I had this hope when she was little. No complaints. She’s good and we are blessed.

Jump to this post

I shared your post with my daughter who is the mother of my grandson with HRHS. I cannot tell you how wonderful it was to hear how well your daughter continues to thrive. We are praying for such a blessing for our dear little Henry. Please keep our baby in your prayers as I will keep your daughter in mine.
Kindest Regards,
Kathleen

@kameg

Kathleen: She is doing very well. She just celebrated her first wedding anniversary this past June. She works full time as a cardiac tech. We are from Philadelphia,Pa and they have a wonderful single ventricle clinic. It was not easy transitioning from pediatric cardiologist, but hers made it a bit easier since he was retiring. Her cardiologist seems to be very pleased with her condition. No liver issues and heart function is doing well. She is a bit blue but not that others really notice. Her oxygen levels are always 89-92 which is her normal. All in all, she’s living a fairly normal life. I say fairly because it’s something I think of and know she really can’t keep up certain paces, but if you just met her you wouldn’t know she had an extremely rough time in the beginning. I hope this helps you. I sure wish I had this hope when she was little. No complaints. She’s good and we are blessed.

Jump to this post

I promise to keep Henry in my prayers. I believe prayers are what brought my Megan to still be with us. God bless

hi, my daughter had a fontan in ’05 in june of 2016 she got a blood clot and at that time we found out that she has cirrhosis of the liver in September we did a heart cath and they said that the liver pressure was at a 17 which I was told should b at 10-12 her heart dr does not know how to release this pressure as far as what medicine can be used for this I am searching for an answer for this cause at this time her liver damage is not permanent however if the pressure stays on there it will be anyone that has idea on this please message me thanks

@erinsmama

hi, my daughter had a fontan in ’05 in june of 2016 she got a blood clot and at that time we found out that she has cirrhosis of the liver in September we did a heart cath and they said that the liver pressure was at a 17 which I was told should b at 10-12 her heart dr does not know how to release this pressure as far as what medicine can be used for this I am searching for an answer for this cause at this time her liver damage is not permanent however if the pressure stays on there it will be anyone that has idea on this please message me thanks

Jump to this post

Hi @erinsmama. We’d be happy to discuss this topic with you. Please send an email to HLHS@mayo.edu or include your email in your reply. Thanks so much and we look forward to hearing from you!

Please login or register to post a reply.
Contact Us · Privacy Policy