The Importance of Liver Health in CHD Patients

A mom and a pediatric gastroenterologist have a heart-to-heart about the need for collaboration in the care of single ventricle patients.

Thank you to Beth Daddario and Dr. Elizabeth Rand, MD, for partaking in this interview.

The Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program includes a multidisciplinary team of experts that specialize in the long-term care of individuals with single ventricle heart defects. It continues to research new ways to improve quality and duration of life for these patients even as liver problems and other complications arise.


The Importance of Liver Health in CHD Patients

Beth Daddario: Hi, my name is Beth Daddario. I'm part of the Family Advisory Council for the Children's Hospital of Philadelphia, and I'm very excited to be at Cardiology 2022. I'm happy today to be with Dr. Liz Rand. Would you mind introducing yourself?

Elizabeth Rand, MD: Yes, thank you. I am Liz Rand and I am a pediatric gastroenterologist, but particularly my specialty is in liver disease. With respect to cardiac children, my role is in the multidisciplinary Fontan Clinic, the Fontan FORWARD Program. Many of these children, in particular, develop liver problems over time, and I help to try to prevent that and take care of it, if necessary.

Beth Daddario: Can you go into a little bit more detail and share why liver health is so important and demands attention in the Fontan circulation?

Elizabeth Rand, MD: Absolutely. Liver is, of course, a very important organ. It is one of the vital organs, without which you cannot survive. But in general, the liver has a few major roles. One thing is that it processes nutrients, makes them more available or more usable by the rest of the body. It also detoxifies anything that has been eaten or drunk that might be harmful to the body.

And so it's very necessary for everything that we eat and drink. In addition, the liver makes a lot of stuff. It makes albumin, the principal protein in the bloodstream and clotting factors, just to name a few. And furthermore, it cleans the bloodstream. So it's filtering the bloodstream continuously and removing waste products, breakdown products, that are formed elsewhere in the body.

Although these are discarded, they're used in a clever way. The liver forms these components into a digestive juice called bile, which then travels into the gallbladder for storage and is released into the intestine when we eat to help dissolve the food. But then whatever's left over and not reabsorbed just ends up in the poop.

So it's a nice way to get toxins out to exit outside of the body.

Beth Daddario: Very good. As a liver specialist, how did you get interested and involved with cardiology and especially the patients with Fontan circulation?

Elizabeth Rand, MD: Well, I believe that it was Dr. Rychik and Dr. Goldberg, together with Katie Dodds, who first approached me about, the issue of liver disease in children with Fontan and their interest in forming a multidisciplinary group to look at these children, to care for them more expectantly and better over time.

And we met as a group together with some other organ specialist, I should say and kind of helped to develop the idea of the single ventricle survivorship program. And I've been really thrilled to continue to be part of that, over many years and participate in multiple discussions and expansions, and I think I've learned a lot about Fontan physiology myself. And I've learned a lot about what happens to the liver in Fontan physiology and how we can mitigate those things. It's been really fun for me to be able to participate. And in large part, that is because I think it's been really successful at helping children and young adults with these unique physiology.

Beth Daddario: Absolutely. What do you feel, from your perspective, has been the most significant advancement in the care of the Fontan patient?

Elizabeth Rand, MD: I was thinking about this earlier, that really, of course, the most significant thing was the development of the process of making the Fontan circulation. And it's such an incredible leap, the idea of doing that.

And especially, I think, as a liver doctor coming from the outside, I'm constantly marveling at that. You know, that all of these children and young adults that we see in the survivorship program, they are very healthy and happy looking members of their communities. And these are children, that before Fontan would easily have died in the first days of life.

So I think we can never get away from the brilliance of that and importance of that intervention. But then I think all of these little nuances, for example, what I do in liver or what's also done by the endocrinologist and immunologist and nutritionist and exercise physiology people. These are each of them equally important finesse points, if you will, that are going to continue to not only extend the life, but improve the quality of that life going forward.

And so it's hard for me to put one over the other, although, of course, I lean towards the liver enhancements.

Beth Daddario: You have to have your favorite, right? What would you like to see in the future care model for the Fontan patients?

Elizabeth Rand, MD: I do think that it's really important, the multidisciplinary approach. And it's really important for the physicians that are involved, because you really do have to gain some expertise in this field.

And it's not for the completely inexperienced hepatologist to just see a child with Fontan every once in a while. So that's really important for the care and for the physicians that are involved or clinicians of all kinds. And working as a group as we do in our now Fontan FORWARD program, it makes it fun and educational for the providers and better care for the patients and their families.

So all of those things together is what really makes it a successful program. And I think that's the most important thing really in all complex disease, not just fun tan physiology, but in all of them.

Beth Daddario: Right. I think you bring up a very good point, though, because it is such a complex diagnosis that unless you've invested time to be able to understand what's involved and understand the patient and the family's perspective, it really isn't a place that somebody could just jump in and substitute.

So I respect you a lot for your thought and bringing that forward because I think that's very, very true, especially in this population.

Elizabeth Rand, MD: Yeah. And one other thing that kind of spins off from that, that I think is important for young adults and families to understand. This is not a separate disease.

I don't want people to kind of walk away thinking, Oh, now my child also has liver disease. It's all part of the same process. And so the fixing of it is also kind of part of the same process. So I think that's really important. Otherwise, it becomes overwhelming for the cardiologists and the families.

And I think it's an impediment sometimes for cardiologists to even refer children because they're worried that it's gonna be frightening or overwhelming to the families. And that there may not be anything much that can be done. But there are things that can be done right. But you may not know that as a cardiologist.

And similarly, as a hepatologist or immunologist or endocrinologist, you may not really understand the to and fro between the organ systems. So that discussion among the providers is especially important in this situation.

Beth Daddario: So you bring up a good point. I'm not sure if we were both in the same session, but one of the physicians today talked about really changing the way we word the communication, you know, instead of surveillance, you know, just changing that into a more positive setting.

And I think what you're saying is true, especially from the family's perspective, is how do we hear that and how do we say it, that it's not another diagnosis. It's not another thing wrong in my situation, but it's just more of a complication that we need to work together to provide the best outcome. So that's very important for families to hear and to understand.

Elizabeth Rand, MD: Yeah. And it's a funny, two way, situation that sometimes the very success of the Fontan physiology, with respect to cardiac output obscures the need for the surveillance of the liver. Because liver congestion can occur even if cardiac output is very good. And so it seems counterintuitive that there could be a problem going on the liver and those.

Those, those two ends, the systemic circulation and the hepatic congestion are independent. And so really you have to go and check for it or you won't notice it. It's asymptomatic. And that's typical of many liver diseases, but I think it's especially difficult to understand for some people in this case.

Beth Daddario: Absolutely. As we close and the last question I'd like to ask is, what advice would you give to the families that you're caring for, especially in the Fontan world?

Elizabeth Rand, MD: One of my most common piece of advice for people in general, is don't freak out. So whatever is going on, we're going to address it, try to prevent development of problems, or if there is a problem, we have a way of dealing with it most likely, right?

And if we don't have a way of dealing with it, we're going to figure one out. That that's kind of what we like to do. A lot of people are worried about diet and other things that they can do to protect the liver and certainly protecting from excessive use of alcohol in the young adults is an important thing, but otherwise there really aren't particular dietary or supplements that I would recommend.

I like to be, keep things simple. But with respect to the liver issues and Fontan, these are manageable things that happen very gradually, so it shouldn't have to be a surprise, and it should be things that we can intervene and prevent some of the difficult outcomes that we might have had, 10 and 20 years ago before this was anticipated.

Beth Daddario: Absolutely. Well, on behalf of the families, I will say they're very lucky to have you on the team, especially on the FORWARD Program. And I want to thank you for taking time out of your busy schedule to join me today.

Elizabeth Rand, MD: Thank you. It was absolutely my pleasure as well.

Beth Daddario: Thanks.

Related Centers and Programs: Cardiac Center, Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program