Ava isn’t letting open heart surgeries stand in her way

Ava doesn't remember her first two open-heart surgeries, which took place before her first birthday. But she won't forget her third heart surgery and neither will her surgeon, Robert Jaquiss, M.D., Division Director of Pediatric and Congenital Cardiothoracic Surgery, Co-Director of The Heart Center at Children's Health℠ and Professor at UT Southwestern Medical Center. That's because the surgery, which took place in 2021, made Ava one of the first patients to receive a groundbreaking heart valve that could lead to a new chapter in medical history.

Patients like Ava – who was born with a heart condition called tetralogy of Fallot – typically need major invasive procedures (open-heart surgery or cardiac catheterization) every few years to replace heart valves which have worn out and stopped working, become infected or simply been "outgrown" by the patient. The new valve that Ava received is designed to transform into an actual, living heart valve which may be more durable, may be more infection resistant and may even grow with the patient.

"A valve like this would be a holy grail because it's designed to grow as a child grows and to last for many, many years. It could be a game-changer for Ava and kids like her," Dr. Jaquiss says.

Overcoming two major surgeries and weeks in the NICU

Ava's parents, Alicia and Ryan, will never forget the overwhelming fear that came with knowing their baby girl had a life-threatening heart defect. And they couldn't have anticipated the strength they would need to get through her first year.

"It took two long surgeries to reconstruct Ava's heart, and we spent weeks in the intensive care unit," Alicia says. "As a mom, it's hard to see your tiny baby dependent on complicated machines. The worst was seeing her struggle to eat. But she was alive, and she was growing, and that gave us hope."

Still, Ava was one of the lucky ones. She received treatment at Children's Health, which is home to a Level IV NICU and some of the world's top pediatric heart specialists. The first new conduit with valve that Ava received lasted more than 10 years – almost twice as long as most implanted valves in children.

"We feel very fortunate – she's had a relatively normal childhood, and she keeps a full schedule with activities and clubs. She's always hungry to try new things, and we love that about her, especially after such a rough start," Alicia says.

Saying yes to a clinical trial – Ava's family wants future heart families to have hope

When Ava's cardiologist, Penn Laird, II, M.D., Pediatric Cardiologist with Pediatric Heart Specialists, a Children's Health Care Network Partner, and Dr. Jaquiss determined that Ava needed valve replacement surgery, they made a plan with her and her parents to schedule the surgery for summer, so that she didn't have to miss school. Dr. Jaquiss also approached the family about participating in a study of the cutting-edge new conduit with valve.

The valve, made by a company called Xeltis, is designed to provide a home for a patient's cells and tissues to migrate into. Over time, it is hoped that those cells and tissues could take over and become a more natural valve.

"This gives us a chance to correct Ava's underlying problem instead of implanting a valve that we know we're going to have to replace in a relatively short time frame," Dr. Jaquiss says. "That means fewer open-heart surgeries and catheterization procedures and less stress for Ava and her family."

Children's Health is the only hospital in the Southwest offering the Xeltis valve as part of this pivotal trial, a necessary step for the valve to possibly gain FDA approval. If the outcome of the trial is positive, leading to FDA approval, this development would potentially change the current outlook for all patients who need cardiac valves, reducing or eliminating the need for multiple open-heart surgeries over a patient's lifetime. However, this hoped-for-outcome requires careful, rigorous evaluation, and the only way to find out if the new valve is truly effective is for families to join clinical trials that test it.

"We never, ever ask families to do something they're not comfortable with," Dr. Jaquiss says. "We explain that a clinical trial might be an option, why it might help them and what the pros and cons are. We also emphasize that no outcome can be guaranteed, and that there is much that is unknown. Patients who take part in such trials choose do so freely and courageously, with the hope that they and others will benefit."

Ava's parents asked Dr. Jaquiss every possible question about the trial. They also thought about all the other parents who have had babies with heart defects.

"Ava is a serious go-getter. We wanted her to have a clear runway with a valve that could last a long time. And we loved the idea of playing a role in research that could give that option to many other kids," Alicia says.

Today, Ava is a healthy aspiring artist

Dr. Jaquiss implanted the new conduit with valve in Ava's heart in July. Today, she's a busy seventh grader who loves art and is planning a career in animation.

"Her imagination has no limits. She can make the most amazing things out of cardboard from the recycling bin and draw beautiful prints on her digital notepad," Ryan says. "She's taken over a room in our house and made it her art studio."

Her parents are also impressed by Ava's compassion for other kids.

"We've had a lot of conversations with Ava about the trial, and she wanted to be part of it because she knew it could help patients like her," Alicia says. "She saw how relieved we were by this valve's potential – it eliminates a lot of fear and worry about another open-heart surgery."

For Dr. Jaquiss, the clinical trial marks another step forward in a quest to raise the bar on heart care for children in Texas and nationwide.

"We do the best we can for every patient, but we also know that today's care is far from perfect," Dr. Jaquiss says. "That makes us even more motivated to improve – we're constantly looking for ways to make care better, so kids like Ava can have the longest, healthiest lives possible."

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The nationally renowned team of pediatric cardiologists, cardiothoracic surgeons and subspecialists at The Heart Center at Children's Health treat the whole spectrum of pediatric heart problems with a commitment to excellence. Learn more about our cardiology programs and treatments.