When a child is born with only one functioning heart ventricle – a condition known as single ventricle congenital heart disease – a series of surgeries can reroute blood flow to help them survive. The final operation, called the Fontan procedure, allows children to grow into adulthood. But over time, this circulation – which depends on blood flowing passively to the lungs without a pumping chamber – can lead to serious complications.

Harma Turbendian, MD, a congenital pediatric heart surgeon at OSF HealthCare Children’s Hospital of Illinois, is working to change that. His innovative research explores how living donor heart tissue could be used to create a pumping mechanism for Fontan patients – potentially improving blood flow and reducing long-term risks.

"There are a lot of people around the world trying to figure out instead of having the blood flow be passive, like after a Fontan procedure, can we put a pump in the area, and help kids with single ventricle congenital heart disease? And could this help mitigate the bad side effects of that type of physiology?" Dr. Turbendian discusses.

Building on a breakthrough

The idea stems from an earlier medical breakthrough called partial heart transplantation, which allows surgeons to use living donor valves from hearts that can’t be transplanted in full. That innovation, first developed by Taufiek Rajab, MD, at Arkansas Children’s Hospital, opened the door to using viable heart tissue for other purposes.

"I said, what if we took a right atrium from a donor and made it into a pumping tube," Dr. Turbendian recollects. "Then we could use that as part of the Fontan surgery, hopefully having a clinical effect." 

This line of thinking led him to develop a technique called the Cavoatrial Partial Heart Transplant (CAPHT). The goal is to transform the right atrium from a donor heart into a pumping tube that could be used during or after Fontan surgery to help blood flow more efficiently to the lungs.

Testing the concept

With support from the team at Jump Trading Simulation & Education Center and research funding from OSF HealthCare Children’s Hospital of Illinois in Peoria, Illinois, Dr. Turbendian began testing the idea using both porcine (pig) tissue and human cadavers. At a research lab in Arkansas, his team connected donor atrial tissue to a cardiopulmonary bypass circuit, an external device that acts like the heart and lungs.

The results were promising.

The right atrial tissue demonstrated pulsatility, the ability to beat, and could even be paced externally with a pacemaker. The team measured an average pulse pressure of 9.2 mmHg (millimeters of mercury) showing that the tissue could generate blood flow on its own.

"When we connected the tissue to the circuit, we were able to show that it is able to pump. We can pace it externally with a pacemaker, and it can generate blood pressure with its pumping mechanism," Dr. Turbendian says. 

These early findings were recently published in the Annals of Thoracic Surgery Short Reports, an international peer-reviewed journal of cardiothoracic surgery. The publication marks a significant step in validating the concept and sets the stage for future studies.

Looking ahead

The next phase of research will test whether the technique can work in living subjects, starting with donor pig tissue transplanted into another pig to see if the tissue continues to function independently. It’s a long and complex process that could take years, but the potential impact is enormous.

If successful, the Cavoatrial Partial Heart Transplant could one day provide Fontan patients, both children and adults, with a new, living pump to support their circulation and improve quality of life.

Why it matters

Worldwide, an estimated 50,000 to 70,000 patients live with a Fontan circulation. While the surgery has saved countless lives, it leaves patients dependent on a passive blood flow system that can cause fatigue, liver problems and heart failure over time.

By reimagining how donor heart tissue can be used, Dr. Turbendian and his colleagues hope to create a new generation of surgical options; ones that don’t just extend life but also improve how the heart works long after childhood.

Video Interview Clips

That’s what jolted Stephanie Thomas awake late one night. Her 2-year-old son, asleep for only a few hours, suddenly let out a scream no parent ever wants to hear.

“I ran into his room, and he was in his crib, arching his back and letting out these awful screams. Then he faceplanted forward,” Stephanie recalls.

At first, Stephanie assumed it might be night terrors. But when the screaming stopped and her son’s breathing grew shallow, her instincts — and training — kicked in.

“It was an instinct to get CPR started right away.”

Stephanie, a clinical dietitian at OSF HealthCare Children’s Hospital of Illinois, had been Basic Life Support (BLS) certified for 10 years. Though CPR isn’t part of her day-to-day job, her muscle memory took over.

“I pulled him out of his crib and told Kris [her husband] to call 9-1-1,” she says. “I put him on the floor, tried to find a pulse, but couldn’t feel any breath movement. I started doing compressions.”

Her quick actions bought her son precious time.

Emergency responders arrived within four minutes, Stephanie says, and the child was rushed to the hospital. Over the next 11 days, he underwent multiple tests, including genetic testing. The diagnosis: Brugada syndrome, a rare but serious genetic heart condition that can lead to sudden cardiac arrest.

Just before being discharged, her son became the youngest child in the world to receive an extravascular implantable cardioverter-defibrillator (EV-ICD) — a device designed to monitor and correct dangerous heart rhythms without the risks associated with traditional internal leads.

A New Reality

Since that terrifying night, the Thomases’ world has changed. Their son has been hospitalized six more times. The first time he screamed again at home, it turned out his EV-ICD had fired — delivering a life-saving shock to his heart. Additional shocks have followed, some occurring during hospital stays.

The couple’s older child, as well as Stephanie and Kris themselves, underwent genetic testing to determine if they also carried the Brugada mutation. It’s a condition that often lies silent until it's too late.

Stephanie credits Sunita Ferns, MD, her son’s pediatric electrophysiologist, for tapping into a national network of experts to help guide his care. Dr. Ferns says Stephanie and Kris’ son is now “married to cardiology,” but is confident in the techniques and technology the Electrophysiology (EP) Lab at OSF HealthCare Saint Francis Medical Center has to treat him.

“We monitor these devices constantly. If we see any arrhythmia in the background, despite the medication he's on, we can offer him other technologies,” Dr. Ferns says. “Ablative technologies can help modify the substrate, which is the tissue that's responsible for the bad rhythm.” 

The Emotional Weight

Now, the Thomases are learning how to navigate life with a toddler who doesn’t fully understand what’s happening to him — but who knows something is different.

“Medication-wise, he’s a champ. He takes his meds every six hours — even in the middle of the night — without complaint,” Stephanie says. “But the hardest part is when he says things like, ‘I can’t use elephant blankie because it shocked me.’ He makes these associations between being shocked and the objects or places around him.”

Kris adds that one of the biggest takeaways from their experience is the importance of having an emergency plan in place.

“Who’s calling 9-1-1? Who’s doing CPR?” he says. “We never thought about any of that until we were in the middle of it.”

A Message for Other Families

The Thomases now speak about the importance of CPR training and being alert to warning signs in children that could indicate something more serious than a bad dream.

Aside from the importance of knowing CPR, Stephanie says another major lesson to be learned is to pay close attention to your children's behavior to see potential warning signs.

The journey is ongoing, but their message is clear: instincts, preparation and community can save lives.

To read more about Stephanie and Kris’ son’s journey, and to learn from his medical team, visit the OSF Newsroom.

Video Interview Clips

Related stories

Parents' fast actions lead to world-first heart procedure

At 94 years old, every day counts for Jim Bier.

The Champaign, Illinois, resident, U.S. Army veteran, and retired map-maker is able to manage things like visiting the store and strolling his backyard gardens – a space resembling the University of Illinois Japan House gardens, which Bier designed. It all comes with less chest pain and easier breathing thanks to a minimally-invasive heart procedure offered at OSF HealthCare.

“I’ve been at home, exercising, building up my legs, and I don't have the difficulties breathing like I did before all this happened back in the garden days,” Bier says. “And so I would say I'm in very good shape at this point.”

Bier pauses and adds with a chuckle, “From my waist up. I still have to work on my legs.”

Approved by the U.S. Food and Drug Administration in 2011, transcatheter aortic valve replacement, or TAVR for short, deals with a thickened aortic valve in the heart that can’t fully open. This reduces blood flow and could mean symptoms like chest pain, shortness of breath, fainting, and fatigue.

Tariq Enezate, MD, a cardiologist at OSF HealthCare Cardiovascular Institute in Urbana, Illinois, explains that the TAVR procedure begins with making a small cut near the patient’s groin. The doctor then inserts a small catheter – “smaller than my little finger,” Dr. Enezate notes – and guides it up to the heart to insert a new valve. This is opposed to the more invasive open-heart surgery, which can lead to a longer and more painful recovery for the patient. Minimally-invasive surgeries like TAVR also avoid the need to stop the patient’s heart.

“Those patients are old. They have a lot of diseases. So, many of them, they are not good candidate for surgery. And the surgery team might think they might not be in good shape and strong enough to handle the stress of open heart surgery,” says Dr. Enezate of potential TAVR patients. “In those situations, TAVR is actually lifesaving.”

For Bier, the lifesaving series of events began on Christmas Eve of 2021. By then, Bier had been dealing with heart problems for nearly a decade and had used a blood thinner after open heart surgery didn’t yield the best results. But a visit to the emergency department that day turned into a week-long stay and the question: What more needed to be done? Eventually, Bier says doctors told him about one last surgery that was needed – “the big one,” as Bier puts it. He needed a new heart valve.

An avid reader, Bier researched the TAVR procedure to allay his concerns.

“I’m a person who goes with the flow,” Bier says. “You talk to any of the nurses who've dealt with me at the hospital. They're saying all the time, ‘Go with the flow.’ That's what I kept telling them all the time. And in that way I'm pretty optimistic about it.”

Still, Dr. Enezate recognizes that even minimally-invasive heart surgery may cause stress. He says the patient’s care team – everyone from the cardiologist to the anesthesia team – meets with the patient and their family several times before the procedure.

After TAVR patients go home, Dr. Enezate explains, they eventually will enter cardiac rehabilitation to rebuild the strength needed to do the everyday activities they love. Other appointments with a provider and medications like a blood thinner are also typical.

“We have a big team of doctors, nurses, nurse practitioners, therapists, social workers, and so many people,” Dr. Enezate says. “Take care of the patient, take care of their family, answer their questions, get the tests, the procedures that they need. And after the procedure, we are on top of things.”

At OSF HealthCare Heart of Mary Medical Center in Urbana, where TAVR recently launched, that team includes Dr. Enezate, Ashraf Al-Dadah, MD, Alaa Ujayli, MD, and Kelli Lamaster, the cardiac program coordinator. Each of them undertook years of schooling and training to be able to successfully oversee operations like TAVR.

“If you asked a cardiologist 10, 20 years ago and you tell them a patient with valve replacement can go home next day, they would think that you're crazy,” says Dr. Enezate. “But now, it is reality.”

Visit the OSF HealthCare TAVR website for more information and to find a provider near you.

Interview clips - Jim Bier

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Previous TAVR stories from the OSF HealthCare Newsroom

Mick Jagger Recovering After TAVR Procedure - April 5, 2019

Forging the Path – TAVR for Congenital Heart - December 10, 2021

New Innovations are the Heartbeat of TAVR - November 3, 2022

Just call Steve Bugelli and Brad Zorns two peas in a pod.

The men walk in to OSF HealthCare Sacred Heart Medical Center in Danville, Illinois, together and make a beeline for the cardiac rehabilitation gym.

After a vitals check, Zorns, of Oakwood, Illinois, hops on a treadmill. Bugelli, of nearby Westville, steps onto an elliptical.

“You good, Brad?” Bugelli asks.

“I’m ready,” Zorns replies.

Ninety minutes and a good sweat later, the strangers-turned-friends walk back out to their cars side-by-side.

Repeat the routine for the other four weekdays, and it’s easy to see why the men have made tremendous strides in recovering from serious cardiac events.

“It's made a difference,” Bugelli says, the conviction in his voice strong. “Right now, I can get up. Before, I was in the scooter. I couldn't get my groceries. I couldn't do anything. Now, I can. I can go in. I can walk.”

“Rehabilitation is great,” Bugelli continues. “The girls work with you. They work with you at your own pace. You're well-monitored. And it's really helped. It changes your lifestyle.”

Cardiac rehab is a typical, but important part of recovering from something like a heart attack, congestive heart failure, open heart surgery or other cardiac event, says Caitlin Hunt, an exercise physiologist at OSF Sacred Heart who helped start the hospital’s cardiac rehab program in 2020. Hunt and her colleagues Brittney Cromwell and Tricia Herman run the program today.

“My angels,” as Zorns refers to the women.

While we think about building up arm and leg muscles at the gym, your heart is a muscle too, and a pretty important one, Hunt says. She explains that cardiac rehab starts as inpatient phase one, when a patient is still in the hospital. It then progresses to outpatient phase two, where patients exercise two to three times per week over a couple months at the rehab gym. Then, patients “graduate” to phase three (also called maintenance program or community fitness). That’s where Bugelli and Zorns find themselves. There, patients chart their own course and come as they see fit.

“What we do is watch their heart rate and make sure it doesn't get up to a certain level for their age,” Hunt says.  “And then as they progress, we just make it a little tougher for them. Increase their speed or increase their [workout] level.”

Herman, a registered nurse, says OSF HealthCare Mission Partners (employees) also educate cardiac rehab patients on things that go along with working out, like nutrition and managing medication.

Herman adds that the mental health benefits mirror the physical ones. Look no further than the friendship of Bugelli and Zorns, who swapped phone numbers early on in their recovery journey.

“I tried to go to gyms before. You just go in there, and all they want is your money,” Zorns says. “They don't care what you do. I had trouble with my heart rate. They didn't monitor any of that stuff. I go and I'd feel worse after I'd come out.”

Not so at cardiac rehab through OSF HealthCare.

 “Some people may not be able to fully walk on a treadmill for 20 minutes where other people do,” Hunt says. “And then they say ‘well, they're doing that so I can do that too.’ They push each other to be better. They know ‘OK, well you're coming tomorrow. OK, I'll come too.’”

“They've already developed, sometimes, friendships in these classes. Or bonds. Because, ‘hey, I have this and you have this.’ They know about each other's families,” Herman says. “It feels so much easier to come into a community to do your fitness program and keep it up.”

Interview Clips - Steve Bugelli and Brad Zorns

Interview Clips - Caitlin Hunt

Interview Clips - Tricia Herman

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