On a Mission

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By Abbey Nash

On a Mission Ares, 4, was born with a severe heart defect in which the left side of the heart is underdeveloped. If you saw Ares at play today, you’d never know he spent much of his young life in the hospital. Always running and laughing, “he’s a bundle of joy,” says his mom, Jennafer, who once wasn’t sure her son would make it to his third birthday.

The smattering of scars on Ares’s small chest shows some of his story: seven heart surgeries, the most recent a transplant. Born with hypoplastic left heart syndrome, a severe congenital heart defect in which the left side of the heart is underdeveloped, Ares needed care immediately following his birth. His doctors initially planned for three reconstructive heart surgeries. He had the first at 2 days old; the second, only five months later.

Just before Ares’s third operation, a series of viruses left his heart too weak to withstand surgery. It began to fail, and Ares was soon listed for transplant.

“This is my first baby,” says Jennafer. “I was devastated, but I didn’t have time to cry. We were on a mission.”

Fortunately, so is Children’s Hospital of Philadelphia.

Advanced Cardiac Therapies for Heart Failure Patients (ACT-HF) is a program in CHOP’s internationally renowned Cardiac Center. Led by Joseph Rosanno, MD, Chief of the Division of Cardiology at CHOP, ACT-HF translates groundbreaking research into innovative clinical care for children with heart failure, like Ares. With state-of-the-art rehabilitation and one of only two heart failure intensive care units in the country, the program provides critically ill children the full spectrum of care through transplant and beyond.

Meeting the challenge

On a Mission Children like Ares have very specialized needs, which CHOP’s Advanced Cardiac Therapies for Heart Failure Patients program provides. Treating advanced heart failure in congenital heart disease is one of the greatest challenges in pediatric cardiology.

While chronic heart failure in adults often results from coronary artery disease and can be treated through medication, heart failure in children is quite different.

"By and large, heart failure in children is caused by a primary problem with the heart muscle, called a cardiomyopathy, or a consequence of having congenital heart disease that has undergone at least one surgery, sometimes many surgeries, and it is just not functioning well over time," says Dr. Rossano.

Because there are no heart failure medications specifically approved for use in children, pediatric cardiologists tend to "borrow" chronic heart failure treatments used for adults. But children are not small adults; the outlook of pediatric heart failure is often much more dire.

“The likelihood of a child needing a heart transplant is much higher than what we would see in the adult population,” says Dr. Rossano. “The likelihood of death is much higher.”

ACT-HF seeks to improve these outcomes by developing medication specifically aimed at preventing and treating heart failure in children.

Building a better bridge

While Ares waited for transplant with a further weakening heart, his team determined that he needed a ventricular assist device (VAD) — a mechanical pump that supports heart function and blood flow to vital organs — to help bridge the gap until his small body was strong enough to withstand another surgery.

But Ares’s heart valves were also deteriorating, which made it nearly impossible to maintain good circulation, even with a VAD. Ares first needed a valve repair.

“It was a very complex surgery,” says Katsuhide Maeda, MD, PhD, Ares’s surgeon and Director of Mechanical Circulatory Support and ECMO at CHOP. He notes that supporting children with single ventricle heart defects on a VAD is challenging, requiring innovative strategies to buy critically ill patients more time as they await transplant.

“With great advancement of surgical techniques, as well as new mechanical circulatory devices, our results are improving,” says Dr. Maeda.

Because VADs small enough for infants are not always readily available, ACT-HF is also “looking for better strategies to develop pumps for this group of children,” says Dr. Rossano.

A dedicated place for dedicated care

Children like Ares with advanced heart failure often spend extended periods of time — including birthdays and holidays — in the ICU.

Says Dr. Rossano, “Their needs for rehabilitation and their nutritional needs, are very different [than those of most patients in the ICU after elective surgeries]. We want to make sure that we can give them the best care so they can rapidly recover — not just make sure their heart is getting better, but their whole body.”

Having a dedicated ICU team ensures heart failure patients receive the specialized care they need. This includes state-of-the-art exercise, in addition to physical and occupational therapy, so their bodies can grow strong enough to withstand transplant.

A mission complete

In February 2023, after spending 17 long months in the hospital, Jennafer finally received the call she had been waiting and praying for: a new heart had been found for Ares.

“I didn’t tell anyone until Ares was actually in the operating room,” says Jennafer. “There are kids who don’t get to come back home or who never leave the hospital. I needed him to come out with a new heart.”

And that he did. Now Ares is a busy 4-year-old who plays soccer and recently started school. He “has come a long way,” his mom says.

To find out how you can support ACT-HF and help children like Ares grow and thrive, contact Geminesse Johnson, Director of Development, at johnsong10@chop.edu.

Hear more about the ACT-HF program from Dr. Rossano and Susan Furth, MD, PhD, CHOP’s Chief Scientific Officer, on her podcast Bench to Bedside.