Kingston’s story: Changing the trajectory of his life with biventricular repair surgery

When Toykeyah (“Toy”) sits on the sidelines of her son Kingston’s T-ball games, she can’t believe how far her son has come.

If you’d asked her 6 years ago if her son would have the strength and stamina to step up to home plate and swing with all his might, she could never have pictured it.

But Kingston’s entire life trajectory has changed, thanks to a state-of-the-art procedure called biventricular repair surgery.

Finding out her son had three congenital conditions

When Toy was pregnant with her third child in 2018, she went in for a routine, 3-month ultrasound.

She expected to leave that appointment with printed pictures of her growing baby to bring home to show her other kids. Instead, she was told that her baby’s heart and other organs had some concerning abnormalities.

Her OB-GYN referred her to a doctor that specializes in high-risk pregnancies called a maternal fetal medicine doctor (MFM) – and that MFM then referred her to a cardiologist.

Toy soon learned that her unborn baby had heterotaxy syndrome which was responsible for two congenital heart conditions: double-outlet right ventricle (DORV) and atrioventricular septal defect (AVSD). Shannon Blalock, M.D., Pediatric Cardiologist at Pediatric Heart Specialists, part of The Heart Center at Children's Health℠, prepared Toy for the road ahead for her baby – a series of staged surgeries over time to help his heart function better.

Then, at 31 weeks pregnant, Toy’s blood pressure spiked high enough that she was prepped for an emergency C-section. Toy was told there was a big chance her baby would come out blue. “When I heard that first cry, almost immediately, I was flooded with relief,” she recalls.

Baby Kingston weighed in at just 2 lbs. 6 oz. and was immediately hooked up to oxygen, a feeding tube and several machines to monitor his vital signs.

Starting down a path of single ventricle repair

In the first few months of his life, Kingston underwent PDA (patent ductus arteriosus) ligation surgery to restrict extra blood flow to his lungs and needed to have excess fluid drained off of his heart at The Heart Center at Children’s Health. His care team there also began to make a plan for his next heart surgery, when he was big and strong enough to have it.

“Kingston’s heart anatomy was very unique. One of his pumping chambers was very small. We decided to start with the Glenn procedure, which is typically the second surgery kids with single ventricle defects have,” Dr. Blalock says.

After Kingston recovered from Glenn surgery, Dr. Blalock wondered if Kingston might be a candidate for biventricular repair, a newer surgical technique that can repair both pumping chambers of the heart, rather than create a workaround for the one non-working chamber, which is what single ventricle repair surgeries do. She’d seen the research and knew that there were just a few hospitals around the country performing this type of surgery.

The larger Heart Center team had doubts about whether Kingston was a good candidate – until Nicholas Andersen, M.D., Director of the Complex Biventricular Repair Program at Children’s Health℠ and Associate Professor of Pediatric Cardiothoracic Surgery at UT Southwestern, joined their team in 2023.

A turning point in his care that felt like a miracle

“Kingston was a kid that people looked at and thought, ‘this is a kid that can never have a normal functioning heart’,” Dr. Andersen says. “After carefully studying his anatomy, I felt confident I could surgically build new tunnels in Kingston’s heart so his blood would flow in the right direction and he’d have 100% normal circulation.”

Over the course of two long, complicated surgeries – that only a select few surgeons in the nation are qualified to do – Dr. Andersen remodeled Kingston’s heart to have two fully functioning pumping chambers.

“Toy was very brave to be willing to try a fairly novel procedure. I’m thankful we have a very open, trusting relationship and that she was willing to put her son in Dr. Andersen’s hands and not have to travel across the country to have the surgery,” says Dr. Blalock.

When Dr. Blalock and Toy saw that Kingston’s oxygen levels were completely normal, they cried together. Their tears fell because Kingston had never experienced normal oxygen levels in his life. But they were also because Toy knew that this surgery had literally changed the entire trajectory of her son’s life.

Living the normal life of a second grader

After spending countless months of Kingston’s early years by his side in a hospital, Toy still can’t believe her 7-year-old son now has a “normal” heart and can wrestle with his siblings and jump for hours on the trampoline in their yard.

“Once we got his circulation rerouted to where he essentially has a normal circulation, normal oxygen levels, two pumping chambers and four valves, Kingston just took off. He was home just five days after the second surgery, which was incredible,” says Dr. Andersen.

Dr. Andersen happily reports that he hasn’t seen Kingston since – a year and a half later – “which is what we surgeons always hope.”

Dr. Blalock, on the other hand, sees Kingston every 3 to 6 months at Children’s Health Specialty Center Tyler for routine echocardiograms and EKGs to check his heart function.

“Kingston is so fun, and a bit of a jokester,” Dr. Blalock says. “He walks down the halls like he owns the place, giving everyone high fives and hugs.”

Toy is in awe of what an amazing and tough kid Kingston is – and so grateful for his bright future ahead.

Learn more

Our Pediatric Complex Biventricular Repair Program is one of only a handful of hospitals in the nation to offer life-altering biventricular repair for the roughly 30% of kids with single ventricle defects who are candidates for it. And The Heart Center at Children’s Health performs more than 600 heart surgeries for kids each year, with consistently top-ranking outcomes.