Advancements in Understanding the Liver in Fontan Patients Part 2

The Fontan Procedure has been used since the 1970s to treat people with a single ventricle physiology -- also known as a univentricular heart. It has also been used in the last few decades to treat hypoplastic left heart syndrome (HLHS) or hypoplastic right heart syndrome (HRHS). Now there are enough long-term survivors of the Fontan Procedure for the medical establishment to know what kinds of consequences having such a radical physiology poses. When the heart's sole function is to pump blood to the body, and it travels passively to the lungs for oxygen, how does that anatomy affect other organs, specifically the liver?Dr. Fred Wu of Boston Children's Hospital is an expert in working with adults with congenital heart defects with liver involvement. Today's episode is a continuation of the conversation he had with Anna last week. In today's program he talks about whether or not Stage IV of the Fontan is actually transplantation, what advice he feels Fontan Survivors and parents of children born with single ventricle hearts need to hear and finally, he gives us his prediction for what the future holds for this unique population.

The Hearts Unite the Globe (HUG) nonprofit organization is happy to welcome our newest Patreon subscribers, David and Ann Corbly to our HUG family.

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spk_0:   0:05
Welcome to heart to heart with Anna, featuring your host Anna Dworsky. Our program is a program designed to empower the CH d or congenital heart defect community. Our program may also help families who have Children who are chronically ill by bringing information and encouragement to you in order to become an advocate for your community. Now, here is an ID Dworsky.

spk_1:   0:29
Welcome to heart to heart. With Anna, I am Enna Gorski and the host of this program we are in Season nine and our weaknesses and his advancements a congenital heart disease. Our show today is Advancements and Understanding. Deliver and Fontanne patients Part two. And

spk_2:   0:45
our guest is Dr Fred Wu. Want to welcome you back to the program? Dr Woo. Thank you so much for the really informative show we did last week. I'm really excited this week to go into even more depth with you.

spk_3:   0:59
Well, thank you so much for having me back again.

spk_2:   1:01
Dr. Boo 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 has received a Sanofi Aventis Fellow Travel Award for Research, a teaching award from the Harvard Medical School Academy Center for Teaching and Learning Excellence and Grant Funding through Boston Children's Hospitals, Innovation and Digital Help Accelerator for research into innovative approaches to the management of patients with single ventricle physiology. We have really been getting deeply into how the liver is effective when you have a Fontane circulation, and we've talked about liver surveillance, a win Fontane Patients should have their livers assist. But now let's talk about the future of liver treatment and Fontanne patients. And I know we've already alluded to a little bit of this when you were talking about liver transplants and heart transplants together. But I'm gonna be real specific here. Some doctors in the past have said that Stage four treatment of hypoplastic left heart syndrome is considered transplant. So should we consider that this Stage four will really mean Ah, heart and a liver transplant?

spk_4:   2:40
I think most people do agree that Ah, Fontane pal e ation is not a final solution. Eventually, anyone who's had a Fontana operation will probably go on to need something else. Whether that's a heart transplants or whether eventually we are able to come up with some sort of replacement for the missing ventricle remains to be seen. But as Faras, the heart transplant versus heart liver transplant, that certainly has been an area of great concern, particularly for heart failure and transplant specialists. So while the results have been someone mixed, some studies have shown worse outcomes after heart transplant for Fontane patients. And some of the problems that have been reported post transplant include excessive bleeding or, in a few cases, full minute liver failure, where, in addition to the severe bleeding, we see buildup of toxic materials like Billy Reuben and ammonia and the blood that deliver normally metabolizes. So that's why transplant centers are very careful to assess the health of the individual as a whole, deliver the lungs, the kidneys, before they determine whether someone is a candidate for heart transplant. It's also why it's important for those of us who see the Fontane patients every year to keep a close watch on their liver health and their other organ health so that we don't miss that window of opportunity and wait until it's too late to have people meet with the transplant specialist. It is important to point out that just having liver disease is not necessarily a contra indication to transplant. So there was one study that was published a couple of years ago by the group at Washington University in ST Louis. And they found that the outcomes and 20 Fontane patients after heart transplant did not differ between those who appeared to have cirrhosis on CT scanning and those who did not. In other words, we still don't know the best way to identify Fontane patients whose liver disease puts them at higher risk of complications after heart transplant. There's a lot of different hypotheses out there, so some people do, ah, heart catherization toe, look at the resistance in the liver. Some people look for evidence of liver function, but none of those hypotheses yet are backed up by any scientific evidence.

spk_2:   4:43
I'm just really happy to know that there were centers out there that are even considering these concerns because I really haven't heard much about it. I'm so thrilled that you're on the show today, Dr Wu, because I know a lot of us old time parents, especially a lot of questions about this, that we're so thrilled to see that our Children are aging. By my case, most of my friends have Children in their twenties, although I do have some friends who Swanton Children are render thirties. And so it feels like this is something that is really pioneering regarding the work that's being done.

spk_4:   5:18
I have some patients that I follow who are in their fifties and sixties who have had their Fontane circulation for well over 30 years. I think the patient that I follow, who had the Fontane circulation for the longest had his operation 1977 and he hasn't had any operation since then. So I really is attribute Thio the work that a lot of these pioneering heart surgeons have done, you know, both Boston Children's Hospital and Children's Hospital Philadelphia and elsewhere really is quite amazing.

spk_2:   5:47
It really, really is. So do we have any data on the percentage of Fontane patients who actually do need a liver transplant?

spk_4:   5:54
Well, again, the difficulty there comes in not really knowing for sure which patients need, Ah, heart liver transplant, in which patients will do fine with a heart transplant alone. Again, very few heart liver transplants are done compared to heart only transplants. So I would estimate that there's probably somewhere between 10 and 20 Fontane patients who get transplanted per year, and probably about 10% of those go on to get a heart liver transplant instead of a heart only transplant. Part of that is because of the availability. You know, there's only a handful of centers in the country that are doing combined heart liver transplants in Boston. We don't do them at Boston Children's Hospital or Brigham and Women's Hospital. So for the patients where we feel that they need a combined organ transplant, we generally have to send them elsewhere. And that's not a very easy thing to do. If you're sending patients who live in the Boston area out to Philadelphia, not everybody has. The resource is to be ableto pack up and move out there or to even get temporary housing out there for an extended period of time. So many of the patients who are not felt to be candidates for heart only transplant may never go on to be even listed for heart liver transplant because they just don't feel that it's a realistic option.

spk_2:   7:04
Oh wow, I didn't even think about that. And is there also a problem with people who are compromised in other ways? Perhaps if they have autism down syndrome, some other kind of syndrome that's involved, that they might not be listed.

spk_4:   7:21
So the process of evaluating patients for transplant candidacy is actually quite complex. And most centers who do transplant have an entire team of professionals who evaluate the patient from cardiac standpoint from a psycho social standpoint, even from a standpoint of resource is and what they're support system is like. So, unfortunately, patients who don't have a strong support system for patients who have psychiatric issues that might create an issue for them once they've had a transplant with getting regular follow up or taking their medications regularly. Sometimes that can be a barrier to getting transplant in the end.

spk_2:   8:01
That all makes sense, but I'm sure you can imagine It is also heartbreaking to parents who might have Children who do have barriers that would prevent them from being listed.

spk_4:   8:12
It is a very difficult thing, and it's certainly not an easy task for the transplant committee as well. I think generally what it comes down to is that unfortunately in our current society, in our current system, there just aren't enough organs to go around. And so a lot of what thes committees air doing is trying to make sure that the organs that we have available get the best possible use. There's a lot of things that people are thinking about to try to improve the number of people that become organ donors to improve the pool of organs that are out there. You know, I was actually just reading that, and I forget which European country was. I think in Denmark or somewhere, they're actually making people organ donors by default.

spk_2:   8:53
I saw that

spk_4:   8:54
don't want to be an organ donor. You actually have to opt out of that system, which is the exact opposite of what we do night. So certainly doing things like that will improve the pool of organs that are available

spk_2:   9:06
right

spk_4:   9:06
and allow us to do more transplants. And hopefully people will be more likely to get a transplant than to die or get so sick that they get taken off the transplant list. But in our current situation, we just don't have enough organs to go around. So that's why we have tohave people to make those very difficult decisions,

spk_2:   9:23
right? I wouldn't want to make those decisions. I'm sure it is just, ah, horrible process to have to go through and especially to be the one to tell people I'm sorry you can't be put on the list. I would not want to have to be that person. So I respect the fact that there is somebody who does have

spk_1:   9:39
to draw the line somewhere. And I think you're right. If we could make pool of available organs bigger, then we would be able to have more people on that list, a huge difference. When I saw somebody of the sea extreme groups growing, I found family just ready to join May. Anyone who is a member of the adult congenital heart defect community can be a guest on our show. We have a great year plan, and we look forward to sharing other interesting topics. Heart to Heart With Poland David Serving a CHD community Wednesdays at noon Eastern

spk_0:   10:23
Anna Dworsky has written several books to empower the Congenital Heart Defect or C H D community. These books could be found at amazon dot com or at her Web site, www dot baby hearts press dot com. Her best seller is The Heart of a Mother, an anthology of stories written by Women for Women in the CHD community and his 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 baby hearts press dot com to find out more. 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 Gorski at Anna at heart to heart with anna dot com. That's Anna at heart to heart with anna dot com Now back to heart to heart with Anna.

spk_1:   11:25
I know a lot of our listeners are parents of young Children with Fontane hearts, and I'm wondering if you can give us a little bit of advice for the parents who have young Children, what they can do to keep their Children's livers as healthy as possible so we can see the fewest number of consequences from

spk_2:   11:42
having a fun Tian heart.

spk_3:   11:44
Sure, it's

spk_2:   11:45
gonna

spk_3:   11:45
sound very much like the advice that I would given adult patient. But it's particularly important, I think, for parents because a lot of the habits that we get into as adults are really ingrained in us as Children. So the things that we talked about earlier really apply here and will really help to have these patients really be able to look out for themselves as they get older. So again, we really encourage parents to allow their kids to be active, and that's in front of a TV and play video games all day. A lot of the newer information seems to suggest that the more active people are as Children and as adults, the better they tend to do in the long term again, we encourage parents to talk to their Children early about things like alcohol which we all know is potentially toxic to the liver. And you want to talk about that before the kids start experimenting on their own or learning about alcohol from their friends and as Children. When you know when your kids get fevers and things like that. Usually parents will look at ibuprofen or Tylenol to try to bring the fever down, and I think it's okay to use Tylenol. You just have to be a little cautious because Tylenol is potentially toxic to the liver as well, so you don't want to overdo it. I would certainly talk to the pediatrician pediatric cardiologists before using Tylenol to see if they think that's appropriate again. It's really just trying to treat your Children as normally as possible, but just making them aware that there are certain things that can affect their health in the long term. And I think the earlier we can get these kids engrained in these healthy habits and learning how to avoid the things that are potentially toxic to the liver. I think the better that their livers will do as they get older.

spk_2:   13:15
I think that's excellent advice, whether you have a kid with a heart defect or not?

spk_3:   13:19
Yeah, absolutely. I mean, a lot of this is really common sense. I think there's certainly no good way for us to avoid some of the basic problems with the Fontane circulation that affect the liver. So again, the few things that we think make deliver more likely to become vibe Roddick are the longer time that you've lived with the Fontane circulation and the higher your pressures are in the veins. And there's not much that we can do about that because it's sort of a basic part of having the Fontane physiology. But there are certainly things that you can avoid Thio. Try not to add any additional insult to deliver over the long term.

spk_1:   13:51
Well, thank you so much, our two roof for gracing us with two different episodes. I feel like we really have a much better understanding of how the liver is effected by.

spk_0:   14:01
We're certainly learning more and more

spk_3:   14:02
about it. As time goes on,

spk_5:   14:04
Come here is why she always needed a lot of attention. She had strokes, even though it's a national inclination to withdraw from the CHD community. I think being a part of it, help me be part of a solution. Heart to heart With Michael Please join us every Thursday at noon. Eastern. I'm Michael even on and I'll be your host as we talked with people from around the world who have experienced the most difficult moments.

spk_0:   14:34
Anna Dworsky has spoken around the world at congenital heart defect events, and she is available as a key note or guest speaker for your event. Go to heart to heart with anna dot com to learn more about booking Anna for your event. You can also find out more about the radio program. Keep up to date with CHD Resource Is and information about advocacy groups as well as read Ana's Weakly plug. Anna wants you to stay well connected and participate in the C H D community. Visit Heart to Heart with anna dot com. Today 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 Gorski at Anna at heart to heart with anna dot com. That's Anna at heart to heart with anna dot com Now back to heart to heart with Anna

spk_1:   15:33
So now I'm gonna ask you to look into your crystal ball and make a prediction for us. Where do you see the future regarding liver treatment in Fontana Patients?

spk_4:   15:44
Well, I want to emphasize that the Fontana operation has really been nothing short of a life changing operation for people born with one functional ventricle. So as we've talked about, it's really amazing to think that thousands of people out there who had less than a 5% chance of surviving to adulthood just 50 years ago are now raising families. And they're working at entertainment, and some of them are physicians, and some of them are lawyers. But I think just like a lot of the surgeries that were once considered standard of care, so I'm thinking about mustard. And sending operations for transposition have now become obsolete as medical knowledge and technology has improved. So the field as a whole is working toward building a substitute for the missing ventricle and people who have single ventricle physiology. And my hope would be that eventually we won't be doing Fontana operations at all anymore, and we won't even have to worry about the liver.

spk_2:   16:35
Wow, that's an amazing prediction. And I have to hope that perhaps some of this research that they're doing with stem cells might be some of the techniques that you're talking about that might make the Fontana necessary. If we could find a way to give everybody a four chamber heart would not be amazing.

spk_4:   16:56
Yeah, a lot

spk_2:   16:57
of

spk_4:   16:57
the research right now is going into mechanical ways to replace the ventricle. So things like ventricular assist device is replacing or taking over the role of the right ventricle that isn't there. But really, the Holy Grail is if we can essentially grow a new venture over people who only have one functional ventricle and course, that's gonna be a little bit farther off. But if you just look back at the last 45 years of what's been going on with stem cell research and genetic research, it's really quite amazing how fast things can progress. So I think there's a very good chance that within our lifetime we're going to be seeing people coming up with some way to replace that ventricle that's basically grown or developed in a laboratory.

spk_2:   17:39
I think it's really amazing what they've been able to do with cloning, and I've been sad to see that there haven't been more research projects that deal with cloning because unfortunately, it seems like it's a political taboo area to go into. But I remember just 10 or 15 years ago that they were working really hard to clone heart valves and wanting to create the scaffolding to build ah heart in the laboratory using a patient's own cells. Do you think that's something that we will see happen?

spk_4:   18:11
Well, as faras valves go we've already seen in the last couple of years people starting to use three D printers for actually printing biological tissue. So once you have that option three D printing a valve out of a patient's stem cells and with some sort of organic scaffolding actually doesn't seem that unrealistic.

spk_2:   18:31
I think it's very exciting to see where we live right now, the day in time that we live right now that first of all that me just heart Mom can talk with the doctor of your brilliance and pick your brain like this and that we can even have this kind of discussion because 50 years ago this would have even impossible

spk_4:   18:48
Oh, no, I say every day that it's an incredible time to be alive and see all the things that are happening with technology and medical technology in particular,

spk_2:   18:56
the knowledge that you doctors air getting is filtering down to as the common people, so that we can become aware we can become better consumers of healthcare and we can be part of the care team. I think that's a huge shift that we've seen have been just over the last couple of decades. Don't you think so?

spk_4:   19:14
Oh, absolutely. I would say that patients and their families are actually some of our greatest assets and advocates when it comes to doing medical research. I can't tell you how many patients will send me emails or contact me on Twitter, volunteering for any studies on Fontane physiology or single ventricle patients that we might be having.

spk_2:   19:32
Wow, that's great. I love that because I'm sure for a long time one of the biggest problems you had in research was that your subject pool was so small that it was hard to over. Generalize are hard to generalize that all

spk_4:   19:45
right, and I do have to say one of the issues is because there is so much interest in people are so invested in trying to improve outcomes for people with Fontane circulation, One of the challenges we run into is that oftentimes there's multiple studies going on at certain centres. And even though the patients are certainly willing, we can't really have patients enrolled in three different studies at once because sometimes the results will actually interfere with each other. So yeah,

spk_2:   20:10
right. Wow, I didn't even think of that. But yes, I imagine that I could be a problem. Wow. Yes, Well, I have one more question for you and that is what advice do you have for fun? TAM patients themselves in order for them to have a high quality of life with minimal liver morbidity

spk_4:   20:28
Well, at risk of repeating myself again, Many patients that I've talked thio are quite worried or alarmed about the issue of cirrhosis and liver cancer. So again, I want to emphasize that Fontane associated liver disease is very different from cirrhosis that you see associate with alcohol and hepatitis. And most patients do very well and have very few complications directly related to the liver disease. But again, that liver disease does have important ramifications for the current long term, so it's still very important to remain vigilant, even though I would argue that there's no need to panic. So what I recommend to all Fontane patients is that they make sure that they get immunized against hepatitis. So if you already have this chronic injury to the liver, you want to try to minimize your risk of having another potential source of injury added. On top of that, it's now routine to immunize Children for hepatitis A and B. But people from my generation typically didn't undergo hepatitis vaccination, so patients should ask their physician if they were vaccinated for hepatitis and be, And if they weren't, that's something that they should ask about. People should consult with their cardiologists, but for the majority of people, exercise is not only safe, but it's a very important part of maintaining their long term health. I would encourage parents to get their kids in the habit as early as possible of maintaining a diet without too much sodium as people who have heart disease. No sodium causes food retention, and that leads to higher Venus pressures, which in turn leads to more have padded congestion and most cardiologists who take care of congenital heart disease patients believe that the higher the Venus pressures are, the more rapid the progression of liver disease probably is going to be. And as Children reach adolescence and adulthood, parents and doctors should have various serious discussions about keeping alcohol usedto a minimum. I rarely tell people that they should completely abstain from alcohol. So for special occasions like weddings and things like that glass of champagne or something here, there probably isn't a big deal. But again, alcohol is toxic to the liver, so alcohol you should be kept to a minimum. And nobody should be smoking anything, whether it's tobacco or marijuana. Because healthy lungs are a crucial part of optimizing the health and Fontanne circulation,

spk_2:   22:37
there's all seem so common sensible. But we know that, especially when you're a teen agers. Teenagers tend to go through a period of thinking they're invincible, and so they think they could do everything their peers do and even know. Maybe their heart healthy piers shouldn't be abusing alcohol or smoking marijuana, especially or doing any of these things are kids our kids, and so they will go through that teenage rebellious period also, But I think it does help for the doctors to be communicating with the patients that not only is this dangerous for your heart healthy peers, but it's so much more dangerous for you. And I like what you said about. You don't have to completely abstain for May because I think the more you forbid somebody from doing something, the more they want it. But to just keep it very, very minimal.

spk_4:   23:25
I think you make a good point. It's particularly challenging for people who are going through adolescence or growing up with congenital heart disease because you're growing up with scars on your chest and you're going to the hospital periodically. And the most important thing for adolescents is fitting in, and often times that leads to pressure to drink alcohol or to experiment with drugs and experiment with tobacco. I mean, that's something that's holds true for anybody, whether or not they have congenital heart disease. But again, I think if we keep the patients and their families educated so that they know what the potential ramifications are, then hopefully battles help steer them towards making the right choices.

spk_2:   24:05
I hope so, too, and I think it really is all about education. Education is power.

spk_4:   24:10
Oh, I absolutely agree with you.

spk_2:   24:12
And if people want Thio have a good quality of life, they're going to need to make some of those difficult decisions. And that's whether your heart healthy or not,

spk_4:   24:21
right? I think one of the things that's unique about taking care of adults with congenital heart disease versus taking care of kids with congenital heart disease is that when you were a pediatric practitioner, I think you're sort of used to telling families and telling kids, You can't do this, you shouldn't do that And it's much more paternalistic when you're dealing with adults, you know you're dealing with people that are making their own decisions, and everything that people decide to do, whether it's going on a plane ride are going. Skydiving for fun is a risk benefit analysis. So what I do as an adult practitioner really is to try to make sure that the patients have all the information that they need so that they can make the best choices. I would say that there are pediatric cardiologists out there that would tell their patients you should never do something like jump out of an airplane because we've worked so hard to get you to where you are now. But I think a lot of adult cardiologists would say that our whole goal of getting you to adulthood was so that you could live as normal a life as possible and have the same choices and options as everybody else. So I certainly don't want to restrict my patients in terms of things that they want to do as a hobby or for fun or for a profession.

spk_2:   25:27
Yeah, but it is a difficult role for you to play in some ways, because most pediatric cardiologists, in my experience becomes so much more than just your doctor. They become a trusted adviser and in some cases, even like a friend of the family.

spk_4:   25:44
Yeah, it is very tough, and you make a great point. That's actually one of the reasons. I think that transition of care is one of the most difficult areas of caring for adults with congenital heart disease, because many times cardiologists have watched these people grow up. They took care of them as babies. They watched him go through high school with her college, get married and oftentimes the cardiologists are just is reluctant to give up the patients as the patients are to move on to another physician. And I'm not saying that every patient, once they hit 21 years of age, needs to see an adult congenital heart disease specialist. I think there are countries, and there are programs where they have a very strict limit on At a certain age, you transition to the adult program. I think that's something that generally has to be somewhat individualized. But again, I think one of the differences with adult congenital heart disease providers versus pediatric cardiology says there's more of a partnership roll with the patients generally than sort of that parental role that the pediatric cardiologists sometimes have. You don't I mean,

spk_2:   26:43
absolutely. I certainly dio we love all of you doctors because it seems like this is a calling for so many of you. I think the number of hours that you put into becoming the specialized doctors that you are shows how passionate you are about this population, and we feel it. We feel that this is much more than a job for you. This is a calling.

spk_4:   27:06
Oh really, is a privilege, I think, to be ableto work with patients and particularly with this group of congenital heart disease patients. Because all of you guys air so invested and so educated and all of you do your reading and your research before you come in, it's a particularly rewarding cohort of patients to take care of.

spk_2:   27:23
Well, that is a perfect note for us to end this episode. Thank you so much for coming on the show today, Dr Woo.

spk_4:   27:30
Oh, thank you. It's been wonderful.

spk_2:   27:32
It has been wonderful. And that does conclude today's episode of Heart to Heart with Anna. Thanks for listening today. Come back next week to hear the road map to success

spk_1:   27:41
for complex CHD survivors With our guest, Dr Gill Baranowski, you can join us on our new top back session immediately following this show on PAL talk. Just look for the hug podcast, chap. Er And remember, my friends, you are not among

spk_0:   27:56
thank you again for joining us this week. We hope you've been inspired and empowered to become an advocate for the congenital heart defect community. Heart to heart with Anna, with your host Anna Gorsky can be heard every Tuesday at 12 noon Eastern time. We'll talk again next week