Joelle’s heart is aglow, despite complex defect at birth

Pregnant with her third child, Staci Devries went in for what she expected to be a routine 20-week ultrasound. When she was told that her new baby would be born with a serious heart complication, it was all she could do to keep her emotions in check.

“My husband was not with me that day, but my two kids, ages 1 and 3, were, so I had to keep it together in front of them,” Staci said. “With two healthy children, you just don’t expect the next one won’t be healthy.”

Their new baby — later named Joelle — was born in September 2019 with an underdeveloped left side of her heart. If surgery was not performed within the next few days, Joelle would not survive because her heart wasn’t big enough to pump enough blood out to the rest of her body.

After birth and stabilization in the Pediatric Intensive Care Unit, Joelle underwent the complicated Norwood surgery on Day 7 of life, which was performed by Dr. Petros Anagnostopoulos, a pediatric heart surgeon who is also surgeon-in-chief at American Family Children’s Hospital in Madison. Additional surgeries and procedures would be needed to encourage growth of her undersized left ventricle (lower chamber) over the coming months and years.

“Normal hearts operate with two pumping ventricles, but Joelle was born with only a single ventricle to do the work of two,” said Dr. Derek Hoyme, Joelle’s UW Health Kids cardiologist. “The high complexity of this condition means these kids typically undergo three surgeries to reconstruct the way the heart pumps blood to the lungs and the body."

Historically, children like Joelle would never achieve normal circulation. They would undergo three surgeries yet still depend on a single ventricle to pump blood. Fortunately, UW Health Kids takes a more aggressive approach whenever possible.

“Whenever possible, our goal is to rehabilitate the smaller ventricle, so it grows as close as possible to normal size,” said Dr. Anagnostopoulos. “The process still requires three surgeries, but the long-term prognosis is better for a normal life because the heart won’t get as tired with two working ventricles instead of just one.”

Only a handful of centers around the country have the capability to successfully complete this approach, known as biventricular conversion. Children undergoing this three-surgery sequence require highly attentive family monitoring at home.

“We have what we call an interstage program to help families like Joelle’s keep a close watch on the child’s weight, oxygen saturation and feedings in between the first and second operations,” said Dr. Hoyme. “It’s really important that families report anything unusual right away so we can evaluate early and intervene if necessary.”

At 7 months old, Joelle was ready for the second in the series of surgeries to gradually increase the use of the left heart, with the goal to have her heart work with two pumping ventricles. She recovered well from the operation, but unexpectedly remained in the hospital because of an unrelated persistent leakage of fatty fluid that developed between her lungs and chest wall. Very few children’s hospitals intervene when this condition — known as chylothorax — persists, so plans were made for Joelle to go to Children’s Hospital of Philadelphia for a procedure.

“The day after the doctors and our family talked about going to Philadelphia, her fluid leakage began to slow and thankfully, it eventually stopped," Staci said. "We can only conclude that Joelle wanted to stay in Madison. We were so grateful because traveling to Philadelphia would have been a huge ordeal.”

Joelle’s heart condition was the DeVries’ primary focus, but after Joelle’s dad, Bram, received a job offer in Oklahoma, things became more complicated.

“Even though the town we’d be moving to was 16 hours away, we still wanted Joelle to go to Madison for any future surgeries or invasive procedures," Staci said. "We love the UW and wouldn’t bring her anywhere else for advanced care. If our insurance in Oklahoma wouldn’t cover her care in Wisconsin, we weren’t going to move.”

Staci spent two weeks on the phone ironing things out with the insurance company. Ultimately, the coverage was approved, and the DeVries family moved to Hendrix, Okla. — population 79 — in February 2021. While the UW Health Kids heart team still manages Joelle's care remotely, her day-to-day needs are met by Children’s Health Medical Center in Plano, Texas, about a 75-minute drive from their new home.

When she turned 2 in September 2021, Joelle started eating by mouth instead of relying on a feeding tube. By April 2022, testing showed that the rehabilitation of her left ventricle succeeded. It was now large enough to take over pumping blood to the body by itself. Surgery was set for June 2, meaning another 16-hour drive from Oklahoma to Madison for the family of six.

“They took her at 7:45 a.m. for surgery and we finally got to see her at 9:35 that evening,” said Staci. “It was a long day, but so incredible to see her come back with normal color and 100 percent oxygen saturation for the first time in her life.”

During the surgery, Dr. Anagnostopoulos restored all of Joelle’s anatomic connections, allowing her heart to function normally.

“Our team will keep a close eye on Joelle as she grows to make sure the left heart works without a hitch,” he said. “She is recovering beautifully and will hopefully never require any more interventions or surgeries.”

With her third big surgery behind her, Joelle will soon return to Oklahoma to resume chasing her siblings Brigham, Edynn and Kameron and enjoying life on the farm.

“You’d never know she has been through all of this when you watch her play,” said Staci. “She has one heck of a personality.”

Despite the physical distance between Oklahoma and Wisconsin, Joelle will always be part of the “family” at American Family Children’s Hospital.

“Nobody else can beat the doctors in Madison,” Staci said. “There’s not enough I can say about this team. Their standards are very high and driving 16 hours is worth it to ensure that Joelle gets the best care.”

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