Kingston’s Journey
“If you met Kingston, you’d recognize immediately that he’s an adorable, social little guy. What you may not realize is Kingston needs to be watched so closely,” said Tracy Coup, APRN, CPNP, a nurse practitioner in the Department of Cardiology at WVU Medicine Children’s.
Tracy was there when Kingston was born – along with many other caregivers. That’s because, before Kingston even came into the world, the WVU Medicine Children’s team discovered Kingston had a severe heart defect.
When Heather, Kingston’s mom, went in for her 24-week ultrasound, she learned that Kingston had hypoplastic right heart syndrome, a congenital heart defect. Kingston had just one ventricle in his heart, which meant he could not effectively send oxygenated blood throughout his body. None of his veins connect how they’re supposed to, Tracy said.
“Kingston has a very complex heart condition he was born with,” says Jai Udassi, MD, director of the Children’s Heart Center and medical director for the single ventricle clinic program. “When we think about how we need to fix these things to get him ready for surgery, there is a lot of planning that has to start early.”
That’s when a multidisciplinary care team jumped into action.
Care from the very first moment
WVU Medicine’s high-risk OB team followed Kingston in utero and began making plans in collaboration with the Heart Center, meeting weekly to discuss and arrange Heather’s delivery. While the team did not know definitively what was happening with Kingston’s heart until he was born, when they could do an echocardiogram, that didn’t stop them from preparing.
When Kingston was born, he had a lot of folks to welcome him into the world, including neonatologists, cardiologists, cardiac intensive care specialists, cardiac anesthesiologists, cardiothoracic surgeons, and social workers.
“Everyone in the room was ready to intervene and stabilize Kingston as soon as he was born,” Tracy said. “This system is designed to ensure that any baby who needs therapy right away has what they need in the delivery room.”
The care team moved Kingston to the Pediatric Cardiac Intensive Care Unit right away, where specialists monitored him to make sure he was stable.
“Babies with these types of conditions don’t make too many stops in different hospital departments,” Tracy said. “We are all part of the delivery process, and after delivery, the baby is moved to the cardiac ICU with the neonatal team delivering care there.”
An emergency response
After Kingston was stable, he was scheduled to receive a stent in the first few days of his life to improve his blood flow. Unfortunately, Kingston’s arterial duct spasmed and collapsed during the procedure, and his physician couldn’t place the stent. At nine days old, Kingston went on ECMO (extracorporeal membrane oxygenation), a machine to keep oxygenated blood moving throughout the body.
The care team was ready to step in. When Kingston was just 11 days old, he had his first open heart surgery with Christopher Mascio, MD, division chief for Pediatric Cardiothoracic Surgery, to place a shunt to allow Kingston’s heart to direct blood better on its own.
“When you do things at this level of complexity, complications can happen, but the team was there to stabilize and provide a high level of care,” Dr. Udassi said. “The true test of the team is when things don’t go well, how you come together to keep the baby safe.”
Heart Center offers continuity of care
After his initial surgery and recovery, Kingston was able to go home, but his care never stopped. Kingston was enrolled in the single ventricle program at WVU Medicine Children’s, which meant a weekly call with the attending physician, weekly in-person check-ups, and daily monitoring of vital signs to ensure a healthy baby. Patients and families have a dedicated cardiologist, nurse, and social worker to support them throughout their care.
“The most important part of Kingston’s care is that the team is dedicated to specific complex conditions and collaborates and listens to other specialties and services,” Tracy said.
Heather knew that, like most single ventricular patients, Kingston would need two open heart surgeries in his first four years of life. He received the first, a Glenn procedure to improve blood flow to the lungs when he was about six months old. Next, he’ll require a Fontan procedure within a few years, but for now, he can officially graduate from the single ventricle program and be transferred to general cardiology for follow-ups.
“We have a graduation ceremony and give them a certificate to celebrate,” Dr. Udassi said.
Patients at the center receive neurodevelopment evaluations to ensure they are on track with their development. If needed, patients can also have physical and occupational therapy in the home, as well as feeding support.
Kingston will require lifelong care for his condition, but the treatments and outcomes for patients like Kingston continue to improve. “Our field of medicine is learning more about better at caring for single-ventricle patients over time. Now, these patients can live full lives,” Dr. Udassi said.
Collaboration is key within the WVU Medicine Children’s Heart Center
Kingston’s care has been on track, with regular visits from a care team who know him. “When we come here, I don’t even have to give his history – everyone knows him,” Heather said. “Everyone works together so well. The communication is incredible: If I call with a question, they call back within an hour.”
That comprehensive, collaborative care is built into the DNA of the WVU Medicine Children’s care teams. “Even as a whole children’s hospital, we know most of our peers, which makes collaboration easier,” Tracy said. “It’s easy for us to call and ask for referrals and help to support patients. We want to be a one-stop place for families so we can care for everything in one trip.”
For Kingston, that collaboration goes back to the team that met him in his first moments of life.
“We have created a comprehensive children’s Heart Center with all the providers who touch complex cardiac disease,” Dr. Udassi said. “That includes cardiology, surgery, anesthesia, intensive care, interventional cardiology, electrophysiology, imaging, and neonatology working under one umbrella with seamless communication. We’re focused on taking care of these babies day and night.”
This team meets twice a day to discuss, in detail, all the patients, managing their conditions and progressing them toward discharge. They also hold weekly meetings to discuss outpatients who will receive surgery in the future.
“Our children’s hospital gives us the space to meet in one location as a focused team and bring the best outcomes for patients like Kingston,” Dr. Udassi said.
What does that mean for babies like Kingston? “I want parents to know, especially kids with single ventricles, they can get the care they need right here in West Virginia,” Heather said. “As a mom, I was convinced I’d have to go out of state, but after sitting down and meeting with everyone, I’m glad I stayed here. If it wasn’t for the care team we have here, I honestly don’t think we would still have Kingston. I’m just excited to see him thrive.”
