Continuing Education for Understanding the Liver in Fontan Patients

Dr. Fred Wu returns to "Heart to Heart with Anna" for an update on the Fontan-Liver connection that we have come to understand in the last ten years. What are the factors that contribute to Fontan-Associated-Liver Disease? Who is at risk? What symptoms are present in patients who might have liver disease? What can we do to prevent Fontan-Associated-Liver Disease?

Tune in to discover answers to these questions and much, much more!

Articles that Dr. Wu mentioned in our episode:

Diagnosis and Management of Noncardiac Complications in Adults with Congenital Heart Disease: A Scientific Statement from the AHA

Fontan-Associated Liver Disease: Proceedings from the ACC Stakeholders Meeting

Evaluation and Management of the Child and Adult with Fontan Circulation: A Scientific Statement from the AHA

You can listen to Dr. Wu's earlier podcasts here:

Advancements in Understanding the Liver in Fontan Patients: Part 1

Advancements in Understanding the Liver in Fontan Patients Part 2 

This episode is not meant as personal medical advice, but rather an educational interview designed to enlighten members of the CHD community and to spark conversation about a topic. Always consult your own doctor or medical team regarding your treatment plan.

Please take a moment to follow us on your preferred social media platforms:

Apple Podcasts

Facebook

YouTube

Instagram

If you enjoy this program and would like to be a Patron, please check out our Patreon page

Transcript

Dr. Fred Wu: 0:00

We do recognize that there's probably more to Fontan liver disease than just the congestion alone. In many of the studies that have been done by different groups, including our own, looking at predictors of more advanced liver disease, the only consistent factor seems to be the amount of time that's passed since the Fontan operation.

Opening Music: 0:16

Hajime's Funk(jazzy music from Home. Tonight. Forever. by the Baby Blue Sound Collective)

Anna: 0:23

Welcome to Heart to Heart with Anna. I am Anna Jaworski and the host of your program. Today's show is"Continuing Education for Understanding the Liver in Fontan Patients" and we're honored to have a returning expert on this topic. Dr. Fred Wu is here to discuss with us this important topic. Dr. Fred Wu is board certified in pediatrics, internal medicine and cardiovascular disease. After receiving his doctor of medicine degree from the University of Michigan Medical School in Ann Arbor, he completed a combined residency program in pediatrics and internal medicine at the University of Minnesota Medical Center and a fellowship in cardiovascular disease. Also at the University of Minnesota. In 2007 he completed an advanced fellowship in adult congenital heart disease and pulmonary hypertension at Harvard Medical School and stayed on to become a faculty member of the Boston Adult Congenital Heart and Pulmonary Hypertension Program. Dr. Wu came on Heart to- Heart with Anna in Season 9 to talk to us about the Fontan heart and liver considerations. He has also spoken about this topic at the Adult Congenital Heart Association's annual conference and other congenital heart defect conferences. Today we'll be talking with Dr. Wu about what researchers have discovered about the Fontan-liver connection, what kind of diagnostic and management tools are available, and about what's next for Fontan survivors. Welcome back to Heart to Heart with Anna. Dr. Wu,

Dr. Fred Wu: 1:46

Thanks so much. You know, I can't believe it's been almost three years since I first came on your show to talk about Fontan associated liver disease.

Anna: 1:53

I know! I can't believe it either. And I think part of that's because we keep running into each other at these other conferences. So I get to hear, at the conferences, what's new but it's about time that we bring it back to the Heart to Heart with Anna show because I've had so many people asking for more information from you.

Dr. Fred Wu: 2:09

Well, I'm happy to be here.

Anna: 2:11

Well, let's get started by having you explain, first of all, why Fontan patients are at-risk for developing liver issues over time?

Dr. Fred Wu: 2:19

Well, our best understanding of it right now is that the liver disease and Fontan circulation is largely driven by congestion due to the increased venous pressure that results after the Fontan operation. In most people, the pressure in the veins is about six millimeters of mercury or lower and it doesn't really change all that much with exercise, but in someone who's had a Fontan operation, central venous pressure is 10 millimeters of mercury or more and that can double during physical activity and the liver ends up being subjected to that higher pressure as well. In addition to that, the cardiac output, which means the amount of blood that's circulated through the body in any particular period of time decreases after a Fontan operation, and those individuals have a limited ability compared to the general population to increase that output during exercise. So these changes in blood flow and blood pressure ultimately result in fibrosis or scar formation in the liver.

Anna: 3:10

Wow. I didn't realize there was that much of a difference with the Fontan heart compared to a heart that hasn't been operated on before. That's kind of scary.

Dr. Fred Wu: 3:20

The difference absolutely is only about four to six millimeters of mercury for the most part. Although as patients get sicker, that number can go up much higher. So one of the things I think that has been a little bit challenging for us is to figure out exactly why these relatively small changes in pressure actually lead to such severe fibrosis in some cases.

Anna: 3:41

Are there any other factors that contribute to the relationship between a Fontan circulation and liver disease?

Dr. Fred Wu: 3:48

Well, we do recognize that there's probably more to Fontan liver disease than just the congestion alone. In many of the studies that have been done by different groups, including our own, looking at predictors of more advanced liver disease, the only consistent factor seems to be the amount of time that's passed since the Fontan operation. Things like the venous pressure or cardiac output, which we've talked about, ventricular function or whether an individual has a right or left ventricle- none of those other variables seem to predict the degree of liver disease. So there must be something else at play, but whether that's inflammation or abnormal lymphatic drainage or maybe insults to the liver at the time of heart surgery, we still don't really know.

Anna: 4:27

It feels like the more we learn, the more we know we need to learn. It's kind of crazy. So have there been any new longitudinal studies that have been able to shed a little bit more light on what is happening with the Fontan heart and its liver?

Dr. Fred Wu: 4:42

As far as longitudinal studies, there have not been a whole lot of studies done yet. Most of the studies that have been done have been cross sectional studies, meaning we look at certain markers of liver function at one point in time, but we haven't had a lot of studies that have gone on to look at how patients do over the course of say five or 10 years. And one thing to keep in mind is that we really only started to recognize that liver disease is a significant problem in this population about 10 years ago. So most of the studies that we're doing now are still fairly exploratory. But I do expect that over time we will start to see whether some of these things that we follow can predict how patients do in the longterm.

Anna: 5:20

And that's one of the important things is that right now we know that there are some people who do experience liver problems, who have a Fontan, but there are some people who have a Fontan and are making it till their 30s and 40s and not having any problems. Isn't that true?

Dr. Fred Wu: 5:35

It is. And one of the important things is when we talk about liver disease, one question that we need to ask is,'What exactly do we mean when we say liver disease?' I think when we talk about this entity that most people are now calling Fontan associated liver disease, we're really talking about any kind of abnormality that you see on imaging studies or on blood tests. That doesn't necessarily mean that people have clinical symptoms, you know, so it's not necessarily affecting their lives in any substantial way. So if we do blood tests and we see that certain blood tests related to the liver are elevated, we may do an imaging study and oftentimes, if not most of the time, we'll see that there is some degree of scar formation. And to us that's considered liver disease. But most of these patients have no symptoms whatsoever.

Anna: 6:18

Right. And we'll be talking about that a little bit more in the program. But for right now, let's talk about prevention. We all know prevention is important. What would you say to parents of young children who have a Fontan physiology? What can they do to protect their children's livers as much as possible while they're growing up?

Dr. Fred Wu: 6:36

There's two parts to this. One of the parts is what we as the healthcare providers are responsible for and the other is the parents and the patients themselves. As cardiologists and cardiac surgeons, it's our job to try to keep the circulation working as efficiently as possible in kids who've had a Fontan operation. What that means is if we see areas of significant narrowing in the blood vessels, we try to open those up or if we see leakiness, severe leakiness of the valves developing, we would try to reduce that somehow. As parents and patients, the most important thing to do is really to try to maintain as active and healthy a lifestyle as possible. We know that being overweight or obese, having diabetes, having high cholesterol and consuming excessive alcohol can all adversely affect liver health. Exercising regularly, maintaining a healthy weight, observing a diet rich in fruits and vegetables, healthy fats and unprocessed foods and keeping alcohol intake to a minimum can all help keep the liver and the heart as healthy as possible. And finally, immunizations against Hepatitis A and Hepatitis B are also safe and important ways to protect the liver.

Anna: 7:38

See, a lot of times we don't talk about that. Keeping our children shots up to date is very important, especially for something like Hepatitis A and Hepatitis B.

Dr. Fred Wu: 7:48

Yeah. Fortunately, you know, things have changed a lot in the last few decades. So back when I was getting my shots, Hepatitis A and Hepatitis B were not things that were routinely considered. They were only used for people that were considered to be at risk. But, uh, I believe in this current generation, children who are getting immunizations will receive at least Hepatitis B, if not Hepatitis A as a routine part of their immunizations. But some of the older patients who may not have received those when they were younger, you know, as they get older, that is something that most of the hepatologists do recommend, especially when they're already at risk for other kinds of liver disease.

H2HwMichael: 8:22

"Texas Heart Institute were offering us a mechanical heart and he said,"No, Dad, I've had enough. Give it to someone who's worthy."""My father promised me a golden dress to twirl in. He held my hand and asked me where I wanted to go.""Whatever strife or conflict that we experienced in our long career together was always healed by humor." Heart to Heart with Michael... please join us every Thursday at noon Eastern as we talk with people from around the world who have experienced those most difficult moments.

BabyHeartsPress: 8:43

Anna Jaworski has written several books to empower the congenital heart defect or CHD community. These books can be found at Amazon.com or at her website, www.babyheartspress.com. Her best seller is'The Heart of a Mother,' an anthology of stories written by women, for women in the CHD community. Anna's other books,'My Brother Needs an Operation,''The Heart of a Father' and'Hypoplastic Left Heart Syndrome: A Handbook for Parents' will help you understand that you are not alone. Visit babyheartspress.com to find out more.

Anna: 9:28

This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. The opinions expressed in the podcast are not those of Hearts Unite the Globe, but of the Hosts and Guests and are intended to spark discussion about issues pertaining to congenital heart disease or bereavement.

Rejoiner: 9:50

You are listening to Heart to Heart with Anna. If you have a question or comment that you would like addressed on our show, please send an email to Anna Jaworski at Anna@HearttoHeartwithAnna.com. That's Anna@HearttoHeartwithAnna.com. Now back to Heart to Heart with Anna.

Anna: 10:08

Dr. Wu, in the first segment you and I were talking about why a Fontan heart survivor may have problems with their liver and how they may not even experience any kind of symptoms, but there could be some symptoms that a patient should look for, especially if they have a more advanced problem with their liver. So can you talk to us about how liver disease might be expressed in Fontan patients as far as symptoms are concerned?

Dr. Fred Wu: 10:36

Right. So as we've discussed, for the most part, the type of liver disease that we see in Fontan patients is asymptomatic at least until the disease reaches an advanced stage. In some cases, the doctor may be able to pick up on enlargement of the liver or spleen when examining the abdomen, but most people wouldn't be able to tell that their liver is enlarged. Sometimes a symptom of congestive heart failure may be abdominal discomfort from increased congestion of the liver or accumulation of fluid in the abdomen. But symptoms of cirrhosis or liver failure, like yellow discoloration of the eyes or skin, confusion and easy bruising are pretty rare and only happen when someone is very sick.

Anna: 11:14

Well, that's good to know because I think a lot of times when you think'liver disease,' that's what you think. You think jaundice and that there, I didn't know about confusion, but that there might be some problems like that, but I guess that's what's associated with cirrhosis from damage to the liver from alcoholism or other problems. Right?

Dr. Fred Wu: 11:32

Exactly. And one of the questions that we get a lot is, you know, people will hear from their doctor that they have cirrhosis and what is the first thing that people do nowadays? They go onto Google and they look up'cirrhosis' and most of what you find on Google pertains to people who have been heavy drinkers, which is the most common reason for liver disease in the United States and in many of those cases patients will look very sick and I think that tends to scare people or parents because their expectation when they hear the word'cirrhosis' is that's what's going to happen to them. But what we see is a very different type of liver disease.

Anna: 12:05

I had noticed since my son was a baby- because he has a Fontan heart- for those of you who are listening and don't know, my son is a single ventricle survivor. He's 25 and ever since he was an infant, I know that the doctor would always feel where the liver was and would let us know if everything was okay down there. But it was just- a lot of it was just done by feeling. Is that still currently one of the ways that they determine whether or not there's congestion in that area?

Dr. Fred Wu: 12:31

Well, physical exam is still a crucial part of our assessment of the patient. And the nice thing about physical exam is that it doesn't really cost anything. So every time we see the patient, it is important for us to not just listen to the heart and the lungs, but to assess the patient overall. Particularly since we know that people who've undergone a Fontan operation, are at risk for other things. So one of the things that we do routinely is we will feel the abdomen, try to determine if there's enlargement of the liver, try to feel if there's any masses that shouldn't be there, try to see if there's any abdominal tenderness, if there's any distension of the abdomen. And of course there's other types of organ disease that can happen as well. So we always look at the legs to see if there's evidence of venous varicoceles because one of my colleagues did a study that shows that there's a very high incidence of lower extremity venous variscosities and people who've had a Fontan operation.

Anna: 13:22

What does that mean? What's a variscosity?

Dr. Fred Wu: 13:25

Basically those are varicose veins. For some of the same reasons that we see liver disease, which is that the pressure in the veins is higher over time, what we often see in people who've had a Fontan operation is that the veins, which see these higher pressures, will start to stretch out. So, you know, oftentimes people tend to think of varicose veins as being something that you have to worry about when you're much, much older. But there are a lot of people who've had a Fontan operation where at a fairly young age they'll have a lot of varicose veins in their legs or they may get things like spider veins and some of those things can put people at risk for potential blood clots. So we need to watch out for that as well.

Anna: 13:59

Wow. Well, liver surveillance and management are vital for Fontan patients. Have there been any advances in identifying and managing liver disease in Fontan patients?

Dr. Fred Wu: 14:09

Most of what's been learned since we first discussed this, Anna, is that there are a lot of limitations to our current tools for assessing the liver. There's been a lot of interest in some circles with a technology called elastography that uses ultrasound or MRI to measure the stiffness of the liver. But unfortunately the congestion that we get because of the Fontan circulation is a serious confounder that makes it difficult to translate measurements of liver stiffness into estimates of liver fibrosis. And one of the areas that we've been focusing on is how to measure liver function rather than just looking at the liver physically or anatomically. And there are a couple of ways out there to do this, including a pretty new technique that's called HepQuant, um, which is very but unfortunately also very cumbersome. So it's similar to the oral glucose tolerance test that some women get during pregnancy except the individual is given both an oral agent and an IV agent and then they have to sit there for 90 minutes while someone draws blood samples at six different time points. There's been one recent study already that shows clear changes in liver function in people who've had a Fontan circulation using that technique, but it's still very new and we're still learning whether it's something that will be useful or practical for people with a Fontan.

Anna: 15:23

Wow, that's really different. That is something I haven't heard of before. I know that when we talked three years ago, we were talking about some of the different ways that pediatric cardiologists were trying to assess patients and we had said there was no specific guideline and many researchers agree that there needs to be a guideline for diagnosing and managing liver disease in Fontan patients. Has there been any progress towards coming up with guidelines that are being used everywhere around the world because it seems like each hospital had its own guidelines for Fontan patients.

Dr. Fred Wu: 15:58

I think that still is true to some extent. Even within a hospital. Every cardiologist sort of has their own way or their own preferred method for screening for liver disease in these patients. But there has been some progress in this area. There've been a few similar but unrelated efforts. So two years ago there was a document published summarizing the findings of a 2015 American College of Cardiology Stakeholders Meeting specifically thinking about Fontan associated liver disease. Now this wasn't a guideline document, but there were recommendations for so-called sound practice with regard to prevention and screening. And then around the same time I cowrote a scientific statement on behalf of the American Heart Association on diagnosis and management of non-cardiac complications, which included liver complications in adults with congenital heart disease. And finally, earlier this year there was a second scientific statement from the American Heart Association on the evaluation and management of the child and adult with Fontan circulation. And again, none of these were guideline documents, but they do represent the first step towards standardizing how we monitor and manage liver disease in our Fontan population. And I believe all of these documents are free access. So I can provide you with links to share with your listeners after this taping if you like.

Anna: 17:10

That would be great. And I'll have the links in the Show Notes so if any of you would like to see that, just go to the Show Notes, and that's the description of the show, and we'll have the links there for you. When you and are were speaking about this several years ago, we were talking about some of the different techniques that were used to assess the liver, and it's like you said, there was the physical, there's MRI, there is CT scan too, if I'm not mistaken. Is that still what's available today?

Dr. Fred Wu: 17:40

And for the most part there's different techniques within MRI and ultrasound and CT scan. So when you send somebody for a CT for example, we don't always just ask for a CT of the abdomen because unless you specify what it is you're looking for, the test that they do may not give you the information that you need. So there are new techniques using existing technologies specifically to look at the degree of fibrosis in the liver and to look for the presence of nodules.

HUG Store: 18:09

Hi, my name is Jamie Alcroft and I just published my new book, The Tin Man Diaries. It's an amazing story of my sudden change of heart as I went through a heart and liver transplant. I can think of no better way to read The Tin Man Diaries than to cuddle up in your favorite Hearts, Unite the Globe sweatshirt and your favorite hot beverage of course in your Hearts Unite the Globe mug, both of which are available at the HUG Podcast Network online store or visit heartsunitetheglobe.org.

HUG Message: 18:44

Heart to Heart with Anna is a presentation of Hearts Unite the Globe and is part of the HUG Podcast Network. Hearts Unite the Globe is a nonprofit organization devoted to providing resources to the congenital heart defect community to uplift, empower, and enrich the lives of our community members. If you would like access to free resources pertaining to the CHD community, please visit our website at wwwcongenitalheartdefects.com for information about CHD, the hospitals that treat children with CHD, summer camps for CHD survivors, and much, much more.

Anna: 19:22

So during the break, Dr. Wu, you and I were talking about how there isn't enough empirical evidence for the doctors to all have the same guidelines. Can you talk to me briefly about what you and I talked about during the break so our Listeners can hear it, too?

Dr. Fred Wu: 19:37

Oh sure. So one of the things we were talking about is why there are no formal guidelines at this point and why is it that every cardiologist that you go see will have their own way of doing things. And really the problem at this point is we're still very much in a data gathering phase. So we're learning a lot everyday about Fontan and how it affects the liver. But at this point we don't really have the studies that show what the best way to monitor for liver health is. And we don't have any evidence as to whether there's specific treatments that are going to slow down the progression of liver disease or improve outcomes. So when you look at guideline documents from the American College of Cardiology and the American Heart Association, we always base that on evidence. So when you look at specific recommendations, they'll tell you how strong of a recommendation it is and how strong the evidence is that the recommendation is based on. And most of what we do at this point is really based on what we call'expert opinion.' So it's people, like me for example, where we see a lot of people with Fontan and we deal with a lot of Fontan liver disease and we make some educated decisions about what perhaps is the best way to go about managing or monitoring these patients. But if you were to ask me,"Do you have any evidence to back up what you're doing to show that what you're doing is the best practice?" You know, at this point there's very limited data. So the hope is that eventually as cardiologists agree on what the best practical approaches right now we'll be able to collect that longitudinal data that you were asking about earlier and we'll be able to show that certain things are useful and certain things may not be so useful and what sort of things we can do that will slow down progression of the liver disease and hopefully prevent cirrhosis or symptoms from happening. And once we get those, then we can make solid guidelines where we tell cardiologists,'This is what you need to do based on the best current available evidence.'

Anna: 21:23

Well, I think what's so exciting right now is that we have a larger and larger cohort of survivors who are entering their 20s, 30s, 40s, even 50s, right?

Dr. Fred Wu: 21:33

Oh absolutely. Yeah. I mean the people that are in their 50s now are generally people that got Fontan later in life. You know, the very first Fontan was really only done in 1967 and I would say that it really didn't get broad pickup until late in the'70s so the majority of our Fontan patients who had the Fontan as children, as infants, are now, you know, around 40 or so. But there are a few patients who are in their 50s and 60s but those are sort of special cases.

Anna: 22:00

And those were people who were not born with hypoplastic left heart syndrome. They were probably born with hypoplastic right heart syndrome or tricuspid atresia, something that wasn't quite as life-threatening as HLHS. Isn't that right?

Dr. Fred Wu: 22:13

Correct. These older patients who've had a Fontan are generally people that had disease where their circulation between the body and the lungs was balanced enough that they were able to survive for a couple of decades without having a definitive surgery. So most of these people had very low oxygen saturations, but they were doing okay and they were able to survive until the Fontan operation became an option for them.

Anna: 22:36

Well, we do know that sometimes people have to have organ transplantation and that sometimes when we have a patient who is getting a heart transplant, they'll look at the liver to decide if they need to have a heart and liver transplant. Is that happening more nowadays where there's actually a heart and liver transplant done instead of just the heart transplant?

Dr. Fred Wu: 22:58

I think that's a very good question. One thing that's worth pointing out is that it's likely that every person with Fontan circulation has some degree of liver involvement, but for younger patients who are being considered for transplant, the large majority only need a heart transplant and if you look at studies from the past two to three years, they've shown encouraging results in that survival after a heart transplant for failing Fontan has improved, compared to over 10 years ago, to the point where- one-year survival at this point, is similar for people who've had a Fontan, to that of heart transplant for other indications. I think what you're asking about, I believe is patients with more significant liver involvement, which tends to be the adult patients. And in that situation, the trend with many centers does seem to be toward a combined heart-liver transplant for most cases of failing Fontan. There was a paper published by the Philadelphia group a couple of years ago and out of 17 referrals for transplant, 10 were deemed eligible. And out of the seven patients who were eventually transplanted, all of them ended up receiving a combined heart-liver transplant. So Philadelphia is one of the centers, one of the three centers in the United States, that has done the highest number of Fontan heart-liver transplants. And the good news is that at the time of the publication, all of those patients were alive at a median followup of five-and-a-half years after the transplant.

Anna: 24:18

Oh, wow. That's extremely encouraging.

Dr. Fred Wu: 24:21

Yes. But we also have to keep in mind that out of the 17 referrals, most of them, you know, 10 of them actually never went on to transplant. So there is still a lot of room for improvement.

Anna: 24:31

So they never went on for transplant because there weren't a heart and liver available.

Dr. Fred Wu: 24:37

Well, the study that was published didn't go into a lot of details. Seven out of the 17 referrals were considered ineligible for transplant. Now, the reasons for that aren't spelled out, but oftentimes we may tell somebody that they're not a candidate for organ transplant because they're just too sick and we don't think that they'll survive the surgery. Or there may be other issues where we think it's going to make it a problem for them to get a transplant. And then out of the 10 that were deemed eligible, there were three that were delisted, meaning that they were listed for transplant for a period of time and then taken off of the list. And sometimes that's a good thing. So we've done that for patients where they've been sitting on the list, but eventually we decide that they're doing so well that perhaps we don't need to consider transplant right now. So they become inactivated. But there's also patients that get sicker while they're waiting for an organ and they may get sick enough to a point where they're no longer a candidate for transplant.

Anna: 25:31

I know. It's really a fine line that you walk between being sick enough to need the transplant and being too sick to be able to have that transplant. Isn't there?

Dr. Fred Wu: 25:39

Right. Absolutely, and I think that's one of the challenges, not just when we're thinking about transplant, but for a lot of different types of surgeries and interventions. You know, you don't want to wait too long because if you wait too long and you do an intervention too late, oftentimes that intervention becomes too risky or there may be some irreversible damage already done that you're not going to get back even after an intervention. But at the same time, you don't want to do an intervention too early because you know, in many cases these interventions have a limited lifespan. For example, if you're putting in a valve, most of the bioprosthetic valves that we have have a limited lifespan. So you don't want to put one in five years too early when you could have bought yourself five years where the patient would have been just fine. So trying to find that balance is one of the most challenging parts of taking care of patients, just in general, not just adults with congenital heart disease.

Anna: 26:30

Absolutely. And so now I'm going to ask you to take your crystal ball out and make a prediction. So where do you see a Fontan patient on the horizon regarding liver treatment?

Dr. Fred Wu: 26:42

Well, as we mentioned earlier, Anna, prevention is really key. So while a lot of us are working on ways to better monitor liver health, other people are trying to improve our options so that in the future fewer patients will develop liver problems in the first place. So there's been some exciting research happening, which I'm sure you've heard about, on pumps and other sorts of devices aimed at adding energy to the venous circulation i n the absence of a right-sided ventricle. And there's other research that's being done using stem cells to allow more patients to potentially be candidates for a b i-ventricular repair instead of having a single ventricle Fontan repair and if somebody has two ventricles that should severely decrease their risk of developing liver disease in the long run.

Anna: 27:26

Right. Oh, I think the stem cell research that's being done is just amazing and if we could reduce the number of Fontan hearts that are out there, I think we would be decreasing the amount of liver disease, as well.

Dr. Fred Wu: 27:37

Absolutely. There's a lot of surgeons are working on other ways to do a Fontan, or other ways to palliate somebody, where they only have really one good functioning ventricle. Even if you can harness a small ventricle that's not really able to handle the entire circulation, but it can contribute a little bit to the pulmonary circulation, even that we think may be beneficial in the long term.

Anna: 27:59

Well, it's good to hear that they're looking at those kinds of options instead of just putting all of our eggs in one basket and that being transplant when we know that there aren't enough organs out there for everybody. What about cloning? I don't hear much about cloning anymore. Are they still trying to develop organs for people from their own stem cells or from their own cells?

Dr. Fred Wu: 28:22

I think the word'cloning' is a very fraught word, so you know when you use that word, people start thinking about these movies where we create a clone of a person just so that we can harvest their organs. And I think that's something that people would be very resistant to but there's a few different technologies. So one of the things that perhaps is most advanced is a technique where people are trying to take a person's stem cells and then using some sort of a matrix or scaffold and then seeding it with a patient's stem cells to grow just a specific organ. And they've been able to do this with cartilage, for example, to reconstruct somebody's ear. There was a researcher who I used to work with in Minnesota, who's now at the Massachusetts General Hospital and they've used a technique where they take the heart of a mouse and they completely decellularize it using a special sort of detergent. And then they reseed it with stem cells and they're actually able to get some heart muscle cells to grow and to beat. And so that's one of the ways that people are looking into creating an organ that can be used for transplant instead of having to take an organ from a donor or another patient.

Anna: 29:24

Right. I remember seeing something on the internet, and this was actually a couple of years ago and they called it a'ghost heart.'

Dr. Fred Wu: 29:30

Exactly, yes. And they've been working on that technology for over 10 years, so it's still far, far from being ready for actual clinical use, but it's certainly very thought-provoking and very exciting research. It's just not something that's probably going to be available for quite some time.

Anna: 29:46

Well, I'm glad that they're not giving up on that and they're continuing to see what other options are available because the more tools we have in our toolkit, the more likely we're going to be able to help the maximum number of people.

Dr. Fred Wu: 29:58

One of these techniques that I'm most excited about is that they're scientists that are basically working on basically chimeras of people and animals and sometimes you hear about this in the news and it's sort of distorted in a way to sort of be sensationalistic. But basically what some researchers are trying to do is to create a strain of pigs, for example, that has some of the genetic material from people so that the pig's heart will have proteins and antigens that are very similar to humans so that we can actually do a transplant from the pig into a human at some point, but not have to deal with as much of the rejection issues as we would if we were just using a standard pig heart.

Anna: 30:37

Well, that's interesting because we already use pig valves in people. So you would think that there's potentially enough similarity between a pig's heart and a human heart that maybe we could use something like this. Well, lastly, what advice do you have for parents of Fontan patients, or Fontan patients themselves, for them to live a high quality of life with a minimal liver morbidity?

Dr. Fred Wu: 31:00

Well, we've already talked about maintaining a healthy and active lifestyle and that's not anything revolutionary, I understand. But it is undoubtedly the best thing that one can do for oneself. But I do want to take the opportunity to say something to your audience, and that goes back to something we talked about earlier. You know, I understand how scary and discouraging it can be to keep hearing about all the different things that people who've already gone through a Fontan operation are at risk for as they get older. It's like the family spent years worrying about the heart and now you have doctors saying that everyone with a Fontan has liver disease and kidney function is usually not entirely normal. And now a lot of people are focused on neurocognitive issues and I know sometimes it feels like it just never ends, but I have to tell you that I've taken care of a lot of adults who've had the Fontan and, as you said, some well into their 50s and most of them really do enjoy a very good quality of life. Sure, they need to see their doctors a lot more often than most people do. But you know what? They travel all over the world. They play sports, they're mothers and fathers, they're scientists and engineers, they're doctors and lawyers, they're entrepreneurs and writers. And I think it's important for parents, and young people especially, to see that there are other people just like them, not just living normal lives, but really doing wonderful things. Your doctors are here to help you live your best life and your part of the bargain is keeping up with your appointments and keeping us informed. And we will be your allies and tackle these issues with you as they come.

Anna: 32:32

And that's what I love about having people like you on my show, Dr. Wu. I feel like we do have an ally in you and that makes a huge difference, but you're right. We have to do our part, too. You doctors certainly are going above-and-beyond with all of the research that you're doing and all of the time- look at all this extra time coming on podcasts like mine, going to these conferences- as far as I know you doctors speak at these conferences and don't charge anybody anything. You're doing it out of the goodness of your hearts and that's to help your patients and to help educate the community. We owe it to you to have a good diet, to limit our alcohol intake, to keep our doctor's appointments and not get lost to follow-up care because it's those people who keep those regular doctor's appointments who are able to catch problems early enough that they don't become humongous problems. Isn't that true?

Dr. Fred Wu: 33:21

Yes, absolutely. And they certainly don't owe it to their doctors, but I believe they do owe it to themselves.

Anna: 33:27

Oh, well see that's a good way of saying it. They do. Well, we do. We owe it to ourselves and that stands for the parents of these heart kids too. We all should be taking our medicine and trying to exercise regularly and eat a good diet. It's really tough when you live in the United States. I don't know if it's this hard in other parts of the world, but the fast food is on every corner and it's so easy to get into a bad habit of not eating healthy, but like you said, eating those fresh fruits and vegetables and not having the processed food, cooking things from scratch, that's what can help us to live healthier lives. So we don't have diabetes, high blood pressure, high cholesterol, and lots of the other problems that we get as we get older. That, really, we could minimize with just some changes to our lifestyle.

Dr. Fred Wu: 34:11

Yes, exactly. Yes.

Anna: 34:12

And yes, I love it that you said that so many of your patients are living high quality lives and I think it's because of the work that you doctors are doing, the work that the patient's parents put into raising them to give them the best chance for life and then the patients themselves. I'm so proud of so many of these adults that I see who are sharing information with other adults through social media, encouraging them to have a healthy lifestyle. There's a whole Cardiac Athletes Facebook group that talk about different ways to exercise and stay healthy. I think this is exactly what we need so that all of us can live healthier lives.

Dr. Fred Wu: 34:46

Right. Absolutely. I agree with you.

Anna: 34:48

Well, thank you so much for coming on the program and talking to us about what's new with the Fontan and liver involvement. This has been fabulous.

Dr. Fred Wu: 34:55

No, then thanks for everything that you do for the CHD community, Anna. Let's do it again soon.

Anna: 35:00

Okay, well that does conclude this episode of Heart to Heart with Anna. Thanks for listening today. Don't forget if you found this podcast helpful, you can be a patron of our program with Patreon. Just look for Heart to Heart on Patreon- and that's patreon.com/hearttoheart. And remember my friends, you are not alone.

Conclusion: 35:20

Thank you again for joining us this week. We hope you have been inspired and empowered to become an advocate for the congenital heart defect community. Heart to Heart with Anna, with your Host, Anna Jaworski, can be heard every Tuesday at 12 noon Eastern Time.