Baby on Hospice Receives Second Chance With Lifesaving Heart Surgery

Heart surgeon combines unifocalization with innovative valve repair to save Oklahoma baby

When Hazel Randolph was 3 weeks old, she was put on hospice and given six months to live by her Oklahoma doctors. Her parents, Loren and Josh, were devastated by the news.

“We thought, ‘This is it. We are never going to get to see her grow up.’ I hadn’t cried until then, but that day I really broke down,” says Loren.

Hazel was born with a rare combination of heart defects, collectively called tetralogy of Fallot (ToF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs). Together, these defects add up to poor blood flow between the heart and lungs, and low oxygen to the brain and body. Without surgery, babies with severe ToF do not survive.

At first, Hazel seemed healthy and fine. Twelve hours later, she started turning blue from lack of oxygen. A week later, she received a stent to keep her pulmonary artery open, and three weeks later her doctors said there was nothing else they could do and sent her home.

“It was hard. We wondered how much time we had with her,” says Josh.

The couple shared the sad news that Hazel was on hospice care with family and friends. Along with words of sympathy, they received a spark of hope. A friend of a friend reached out and told them to go to Stanford Medicine Children’s Health.

“She said her child was given the same diagnosis, and Stanford Children’s helped save her,” Loren says.

The couple researched Betty Irene Moore Children’s Heart Center at Stanford Children’s. They learned about Frank Hanley, MD, who decades ago pioneered unifocalization—a heart-lung surgery that repairs all four defects, often at once. Their hope grew. Maybe Stanford could save Hazel’s life, too.

“Stanford Children’s is the number one referral center in the world for treating tetralogy of Fallot with MAPCAs,” says Michael Ma, MD, chief of pediatric cardiac surgery at Stanford Children’s. “Our unifocalization treatment is the gold standard.”

The Pulmonary Artery Reconstruction (PAR) Program at Stanford Children’s is one of a few places in the nation that have a robust team of skilled heart surgeons who perform unifocalization. The team consistently achieves survival rates of 98%, despite caring for children with the most challenging heart defects.

They help train heart surgeons from around the world on the surgery in hopes of saving as many children with ToF as they can.

Coming to Stanford Children’s for tetralogy of Fallot heart surgery

Josh and Loren reached out to Stanford Children’s and shared Hazel’s imaging tests and health history. They were told she was a perfect candidate for unifocalization. When Hazel was 5 months old, they flew out for surgery. Unfortunately, it had to be canceled when the couple tested positive for COVID-19. Their faith kept them strong, but it was yet another hurdle for the family to face.

“Hazel’s oxygen levels were dropping, and she was approaching her six-month life expectancy. We were getting worried,” Loren says.

A new heart surgery date was set when Hazel was just over seven months old. This time it went as planned.

Her unifocalization surgery involved heart repairs (patching holes and fixing valves), along with reconstructing arteries and blood vessels. Collateral (extra) arteries, called MAPCAs, were used to create a functioning pulmonary artery to carry blood from the heart to the lungs.

The operation took about 12 hours. Hazel had a full repair, and she wouldn’t need a second surgery.

“In roughly 90% of patients we achieve a full repair, and in the majority of our patients we can complete that in one surgery,” Dr. Ma says.

The couple was at dinner when they got the call that surgery was done and Dr. Ma wanted to talk with them. They rushed back, and he told them exactly what they needed to hear: Hazel did great.

“Dr. Ma not only has the skills, but he really cared about our family,” Josh says.

Hazel’s unifocalization surgery was successful, but not typical. It involved another step, making it even more challenging. Instead of having four known heart defects to fix, Hazel had five. Her aortic valve was also not working properly.

Discovering another heart defect in her aortic valve

“Unifocalization is a complex operation, but it is relatively routine for us at Stanford Children’s, given the high number of patients we treat each year. With Hazel, the trickier part was repairing her aortic valve, which was failing,” Dr. Ma says.

The aortic valve controls blood flow from the left side of the heart to the aorta, the large artery that carries blood to the body. It’s rare for children with tetralogy of Fallot with pulmonary atresia and MAPCAs to also have a defective aortic valve. An unrepaired aortic valve can lead to heart failure. Fortunately, Dr. Ma is pioneering new and improved ways to repair aortic valves in babies, which empower surgeons to customize operations to a child’s exact needs, resulting in better heart function and fewer repairs down the road.

Dr. Ma partnered with the Stanford School of Engineering’s Cardiovascular Biomechanics Computation Lab, led by Alison Marsden, PhD, and scientists from Betty Irene Moore Children’s Heart Center’s Basic Science and Engineering Initiative (BASE) to create advanced technology to design surgical repairs of very complex heart defects, including aortic valves. Hazel was a perfect candidate for this new, multidisciplinary surgical approach.

“Together, we created a 3-D design of Hazel’s aortic valve, which included a complex computational fluid dynamic simulation of the way her valve opened and closed, and the ideal geometry of her leaflets (flaps that make up the heart valve),” Dr. Ma says. “In surgery, we reshaped her aortic valve to match the simulation.”

Alexander D. Kaiser, PhD, applied mathematician and computational scientist, has been a crucial player in helping to develop the groundbreaking modeling method that meant improved care for Hazel. “It is deeply satisfying to hear how my work impacts real lives after many years of working on the science,” he says.

Dr. Ma used Hazel’s own tissue (versus artificial material) to repair her valve for a better outcome. “When we use nonnative tissue, it often deteriorates quickly. It would have meant many more surgeries in Hazel’s near future,” he says.

Hazel is not expected to need another heart surgery anytime soon. If she does, it will likely be much later in life. The world-renowned Pulmonary Artery Reconstruction (PAR) Program was able to turn Hazel’s dire prognosis into a real chance at life and give her parents the joy of watching her grow up.

“No parent should outlive their kids, and Dr. Ma and the Stanford Children’s team saved Hazel’s life. She is thriving and living her best life, and we are so thankful for them,” Loren says.

Stanford Children’s received recent federal funding to advance their 3-D simulation technology. Dr. Ma and his colleagues are publishing their research, which will feature Hazel and four other babies who benefited from computational simulation in facilitating complex aortic valve repair.

“This was a particularly rare case that leveraged one of our top well-known skills with what I hope will become one of our next well-known skills. We provided care that she could have only received here or possibly in a few other heart centers in the world,” Dr. Ma says.

Hazel stayed close to a month in the hospital before going home. In a few months she will return to Stanford Children’s for a cardiac catheterization to look at her heart and measure its function.

The PAR Program at Stanford Children’s stays connected to their surgery patients, even those from another state or country. To prioritize this, the program has been expanding its team to include more cardiologists and advanced practice practitioners, which will ensure continual monitoring of their patients’ health throughout their lives.

“Every patient we care for is a patient of ours for life. We stay involved and provide care over time to ensure that these kids stay on track,” Dr. Ma says.

Turning 1 and growing every day

Hazel’s family recently celebrated her first birthday with family and friends.

“We are over-the-moon ecstatic! There was a time we didn’t think we would celebrate her first birthday,” Loren says.

Even though Hazel isn’t quite where most 1-year-olds are developmentally, she is catching up and growing every day. She smiles and claps. She waves and says “Mama.” She has more energy, and her heart is working close to normal.

“She is such a happy baby, and she doesn’t need oxygen, which is fantastic,” Loren says.

The couple hopes other families are encouraged by their story of how they came halfway across the country, spent hours with insurance companies and doctors, and found ways to manage care for their older child in order to give their baby a chance at life. They believe that if they can do it, others can too.

“We kept pushing through, and it was definitely worth it in the end,” Josh says. “We hope our story inspires others to be persistent, get a second opinion, and have faith.”