July 7 : In February of 2023, Lisa walked into her local maternal-fetal medicine center for a follow-up ultrasound.
She was just over 20 weeks pregnant with her second child, a boy. While a recent anatomy scan had hinted at issues with her baby’s heart, she’d been told it could just as easily be nothing.
Instead, she left with news that would change the family’s life forever: Her son had hypoplastic left heart syndrome , a rare and life-threatening form of congenital heart disease where the left ventricle – the strongest chamber of the heart – is severely underdeveloped and can’t pump enough oxygen-rich blood into the body.
Essentially, he would be born with half a heart.
Lisa could barely process what she’d just learned. ‘I don’t know what any of this means,’ she thought. ‘Will he even be able to live?’
Finding the answers to her questions felt hopeless – until she and her husband were referred for a second opinion at Children’s Hospital Los Angeles.
While smaller medical centers may rarely see single ventricle heart defects like HLHS, CHLA’s Heart Institute is home to advanced programs designed to give every child with a complex congenital heart condition the best possible chance at a healthy future.
For families who receive a prenatal diagnosis, maternal-fetal medicine experts in the hospital’s Fetal-Maternal Center are often their first point of contact, helping to coordinate comprehensive, streamlined care for expectant mothers and their unborn babies.
Lisa recalls her first phone call with Nurse Care Manager Sheila Perez, RN, BSN: “That was when my perspective flipped. Here was this whole team of people, ready to care for us. I felt like someone was finally hopeful for us.”
Welcoming Beckett
Four months after that call, Lisa gave birth to her son Beckett.
Today, Beckett is an energetic 3-year-old – lovingly described as a “spitfire” who roughhouses with his big brother and enjoys any ride he’s tall enough for at Disneyland.
To a stranger, Beckett may look like the average toddler. In reality, he’s already had two open-heart surgeries, spent 149 days in the hospital, and will likely undergo another major procedure in the next year.
While scientific advances have greatly improved what it means to live with HLHS, the condition requires a three-staged surgical reconstruction, regular monitoring, and lifelong multispecialty care to manage complications.
“It’s been a hard journey—there was a time I thought I’d never smile again,” Lisa reflects. “But it’s also been beautiful in a lot of ways. I’m so proud Beckett is my kid.”
Seeking the experts: Fetal and neonatal cardiology at CHLA
Before Lisa was referred to CHLA’s Fetal Cardiac Clinic, she’d had a hard time understanding what life for Beckett – and her family – would look like after he was born. Her first appointment with Shuo Wang, MD, a cardiologist in the Fetal Cardiology Program, gave Lisa the perspective she needed.
“I went in not wanting to know any statistics, but actually, hearing about CHLA’s strong surgical volumes and outcomes helped,” says Lisa.
“As a pediatric academic medical center, our team is often the first to know about and implement new medicines, interventions, and tools that greatly improve the management of these conditions,” Dr. Wang explains.
Three statistics stood out to Lisa: CHLA’s cardiothoracic surgeons perform more than 700 surgeries on average each year. One in five of those heart surgery patients are newborns. And more than half of neonatal surgeries are high-complexity cases.
“Hearing those numbers provided a lot of hope going into a scary season,” says Lisa.
“Good counseling for single ventricle heart defects comes from an abundance of experience – and following patients long-term to understand what outcomes look like today,” adds Dr. Wang. “We do our best to empower families with the information they need to make their own decisions around their child’s care.”
A plan for Beckett
As Beckett’s due date grew nearer, Lisa and Beckett’s care team arranged Lisa’s care with a neighboring delivery hospital and helped coordinate the intricate series of events following delivery.
Inside the womb, Beckett’s body was getting all the oxygen-rich blood it needed from the placenta. Oxygenated blood reaches the fetal heart via a special blood vessel, which typically closes on its own once babies are born. Then, the baby’s heart and lungs take over.
But since Beckett didn’t have a healthy left ventricle, his heart wouldn’t be able to support him on its own once that closure happened.
The minutes and seconds after his birth would be life-defining.
A group of subspecialists devised a plan to immediately stabilize the newborn using prostaglandin medication – the same hormone that flows through the placenta – to keep the fetal blood vessel open. This would allow Beckett’s heart to bypass his nonfunctioning left ventricle and force his right ventricle (the heart chamber responsible for pumping blood through the pulmonary artery to the lungs for oxygen) to do the job of two.
This solution was temporary, however: The heart can only sustain this setup for a short period of time before risking exhaustion, unstable blood flow, and even heart failure.
Surgery for Hypoplastic Left Heart Syndrome
After receiving detailed imaging of his heart, Beckett moved to the Cardiothoracic Intensive Care Unit where he’d await the first of the three staged reconstruction surgeries: the Norwood procedure.
During the Norwood, surgeons close off the fetal blood vessel and rebuild the heart’s architecture to allow the right ventricle to pump blood to both the lungs and body. Newborn hearts are incredible fragile and small – no bigger than a walnut – which is one of the reasons the Norwood is one of the most complex, high-risk surgeries in existence.
“The fact that cardiothoracic surgeons at CHLA sometimes do multiple Norwood procedures a week was really reassuring for us,” Lisa says.
John David Cleveland, MD, Associate Chief of Cardiothoracic Surgery, explains how the team approaches decision making for each patient: “Any case that’s going into the operating room gets reviewed by our entire institute. A few days before each surgery, 3 to 4 cardiothoracic surgeons and more than 20 cardiologists of different subspecialties meet to align on a plan.”
Typically, the Norwood happens about a week after birth, but Beckett was healthy enough to receive it at two days old. “Every child with HLHS is different. Each surgical approach is tailored to their unique anatomic and physiologic needs,” Dr. Cleveland explains.
Clinical support during the interstage period
The time between each surgery, called the interstage period, can be exceptionally risky – especially after the Norwood.
Even for fragile newborns, years of scientific evidence point to the critical importance of babies spending as much time at home as possible. That’s why the Heart Institute established a specialized interstage monitoring program staffed by three full-time nurse care managers who meet with families regularly to monitor weight and oxygen saturation, while teaching parents how to monitor their child’s vitals at home.
“I was so thankful for the interstage program during that time,” Lisa says. “I called them so often, whenever I had a question.”
Dr. Cleveland explains that the connection between Beckett’s heart and lungs, his pulmonary arteries, were not growing quickly enough to provide his body with the oxygenated blood it needed to function properly.
In the early months of Beckett’s life, he spent more days in the hospital than at home – including a harrowing stay that required interventional cardiologist Darren Berman, MD, to perform an emergency catheterization procedure to open his pulmonary artery.
Not long after, his care team determined he’d need to remain in the hospital until the second of the three procedures: The Glenn.
A complex Glenn procedure
The objective of the Glenn is to reduce the workload of the heart, connecting one of the body’s largest veins – the superior vena cava – directly to the pulmonary artery.
Because Beckett had undersized pulmonary arteries, he was considered a particularly high-risk patient, Dr. Cleveland explains. This required the surgical team to place additional shunts that would allow more blood to flow into his lungs and encourage his pulmonary arteries to grow.
“Our surgical approach for Beckett is not typical for the Glenn, but it made sense for him,” says Dr. Cleveland.
Hopeful days ahead
After additional hospitalizations following his Glenn procedure in January 2024, Beckett has managed to stay healthy out of the hospital.
He regularly meets with Dr. Berman and a multidisciplinary team of CHLA specialists to ensure his heart, brain, and body continue to develop adequately, and to gauge his readiness for the third and final procedure, the Fontan.
For Dr. Cleveland and the Heart Institute team, offering treatments that allow kids like Beckett to enjoy being kids is a major win. Still, they continue to pursue a future where management for HLHS is even safer, less invasive, and more effective.
One way the team is advancing HLHS research is by participating in a global consortium- Opens in a new window exploring the potential of umbilical cord blood to help kids’ hearts grow more effectively after the Glenn procedure.
“While our field has made incredible advances for HLHS, I hope that someday we have a better answer for these kids,” Dr. Cleveland reflects. “We continue to explore opportunities to do so.”
“It’s not an easy or comfortable path for families choosing to move forward with single ventricle surgeries,” he continues, “It is a wonderful testament to grace, mercy, and love imparted to a baby who hasn’t even been born yet.”
“A life we once only dreamed of”
Recently, Lisa and her husband Chris teamed up with another CHLA family to raise funds for the hospital during the 10th Annual Walk & Play L.A.- Opens in a new window event.
On their personal fundraising page, Lisa writes:
“Beckett’s journey began before he was even born, when our prenatal care team at CHLA gave us the hope and strength we desperately needed… Since then, CHLA has quite literally saved his life… caring for him through every step with unmatched expertise, compassion, and love.”
“They didn’t just treat Beckett’s heart,” she continues, “They cared for our whole family and gave him the chance to grow, play, and thrive. Because of CHLA, Beckett has the life and quality of life we once only dreamed of.