Newswise — Iron deficiency usually signals anemia. But in kids with a Fontan circulation, Children’s Hospital Los Angeles researchers found something surprising: Nearly half have iron deficiency without anemia, a hidden problem linked to poorer exercise performance and oxygen delivery.

The finding came out of research from CHLA’s Fontan Program—one of the largest such programs for kids in the country. Led by Andrew Cheng, MD, a pediatric cardiologist in the Heart Institute, the team provides comprehensive multidisciplinary care for children who have had the Fontan procedure—the last of three surgeries that enable patients with certain heart defects to live with one ventricle instead of two.

We spoke with Dr. Cheng about the team’s latest research, the toughest challenges in Fontan care, and the first question every family asks him.

What are you learning about iron deficiency in these patients?

Just looking at standard labs, you wouldn’t think that these patients have iron deficiency because they do not have anemia. But based on our findings, we now actively look for this in our patients.

Our research began with our own large population of Fontan patients, and now we are using data from the Fontan Outcomes Network to better understand the relationship between iron deficiency and exercise capacity in Fontan patients across the lifespan. As far as I know, we’re the only group studying this issue in children.

How are you working to predict which patients are at higher risk for Fontan failure?

We are developing an imaging biomarker. Using MRI, we’re studying how blood viscosity affects pulmonary blood flow—a key factor because in the Fontan circulation, the heart doesn’t pump blood through the lungs. Early findings suggest that thicker blood flow is linked to reduced exercise capacity.

This also relates to our iron deficiency research. One of the primary roles of iron is making red blood cells, and one determinant of blood thickness is how many red blood cells you have.

What are the biggest challenges in caring for these patients?

There is a lack of targeted treatment options. Standard heart failure medications don’t work as well for Fontan patients because the underlying disease is very different.

But the biggest challenge is deciding when it’s time for a heart transplant. You don’t want patients to undergo a transplant unless they really need it. But if you wait too long, they may be too sick to be eligible.

This is a tricky condition to manage, and there is a lot of gray. Sometimes patients feel OK, but their organs are struggling much more than anyone realizes.

Why is multidisciplinary care so important for Fontan patients?

The Fontan circulation affects multiple organ systems. In the Heart Institute at CHLA, our program brings together specialists in Cardiology, Hepatology, Nephrology, Pulmonology, Nutrition, Psychology, nursing, and Social Work.

Families see all of us in one coordinated visit, and we discuss cases as a team. That allows us to make the most informed care decisions, based on the full picture of how the child is doing. These patients also require lifelong follow-up, so our Adult Congenital Heart Disease Program, which is led by Brynn Connor, MD, is dedicated to helping our patients transition to adult cardiac care.

You’re part of a team developing an artificial right atrium for Fontan patients. How would it work?

It’s very difficult to bridge Fontan patients to heart transplant. The artificial right atrium is a project led by Cynthia Herrington, MD, in collaboration with the USC Viterbi School of Engineering, to solve that problem. This would be an off-the-shelf device that would essentially adapt a standard ventricular assist device to support patients with a Fontan while they are waiting for a heart.

We have a patent, and we’re testing our prototype in the lab before we move into preclinical animal studies. It’s exciting because it could have a tremendous impact on patients’ lives.

What is your hope for the future?

We have made tremendous progress in helping many children with single-ventricle defects survive into adulthood. But still, one of the first questions many families ask me is, “How close is my child to needing a heart transplant?”

I don’t want transplant to be the first question on people’s minds. My hope is that over time, Fontan care will shift from managing decline to ensuring that all children survive—and thrive—for a lifetime.

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