If you watched 2-year-old Hayden Stevens throwing balls and climbing on the equipment at his favorite play place, you would never guess that he was born with a serious heart defect that demanded two surgeries and several procedures all before his first birthday.
“He’s extremely athletic. He already loves sports—baseball, soccer, and basketball,” says Kelli Jensen, Hayden’s mom.
At 15 weeks into her pregnancy, Kelli and her husband, Dan Stevens, received the devastating news that their baby’s heart wasn’t forming correctly, but it took another 10 weeks of visiting various specialists near their home in San Diego to pinpoint exactly what congenital heart defect Hayden had: tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries, or MAPCAs.
“We were advised to terminate the pregnancy, but I did my research and I learned about Frank Hanley, MD, at Stanford Medicine Children’s Health. I knew he pioneered a surgery for this condition,” Kelli says.
Tetralogy of Fallot (ToF) is four congenital heart defects in one: narrowing of the pulmonary artery (the artery that carries blood from the heart to the lungs), a ventricular septal defect (a large hole in the wall between the two sides of the heart), an overriding (misplaced) aorta (the artery that carries blood to the body), and a thickened lower-right side of the heart. All of these defects add up to poor blood flow between the heart and lungs and less oxygen-rich blood going to the brain and body. Without surgery, babies with severe ToF do not survive.
The couple reached out to our Betty Irene Moore Children’s Heart Center and had a virtual second opinion consult with Dr. Hanley in the Pulmonary Artery Reconstruction (PAR) Program and Michelle Kaplinski, MD, in the Fetal Cardiology Program.
“That conversation still sticks in my mind. Dr. Hanley said, ‘I can fix this. There’s an 80% chance your child will live a happy, normal life.’ They were so confident, which was just what I needed,” Kelli says.
A remarkable surgery that gives kids with ToF and pulmonary atresia a future
Not all babies with ToF have pulmonary atresia, but Hayden did. In pulmonary atresia, the valve that allows blood to flow from the heart to the lungs doesn’t form. Because blood can’t get to the lungs, a collection of collateral (extra) arteries, called MAPCAs, form in utero to do the job, instead. These extra arteries branch directly off of the aorta or other vessels in the body. They carry blood to the lungs so it can be oxygenated.
It’s these collateral arteries that are behind the novel unifocalization heart surgery that Dr. Hanley pioneered and brought to Stanford Children’s in the mid-1990s. Today, Lucile Packard Children’s Hospital Stanford, the core of the Stanford Medicine Children’s Health network, is known across the country and parts of the world for its long-standing expertise in unifocalization, the flagship surgery of the Pulmonary Artery Reconstruction Program. PAR at Stanford Children’s is the only place in the nation that has an entire team of skilled heart surgeons who perform unifocalization. As the national and international leaders in pulmonary artery reconstruction, we provide the surgery two to three times a week, giving children like Hayden a near-normal heart that lets them grow and be kids.
“In ToF with pulmonary atresia, every child’s collaterals (MAPCAs) are different. They might have three or they might have nine. They might originate in the chest, the abdomen, or the neck. During unifocalization surgery, we take these collaterals and create functioning pulmonary arteries,” Dr. Hanley says.
After hearing Dr. Hanley’s hopeful diagnosis, Kelli knew that she wanted Hayden to receive care at Stanford Children’s. She had grown up in the Bay Area, so they were able to stay with her parents to be close to the hospital.
A supportive prenatal-care journey and birth
Kelli received prenatal counseling and continued care from Fetal Cardiology, which included echocardiograms (ultrasounds of the heart) and genetic testing to learn everything they could about Hayden’s condition. She received advanced obstetric care from a maternal-fetal medicine specialist, Katherine Bianco, MD.
“Maternal-fetal medicine doctors are high-risk specialists, trained to help with complicated pregnancies,” says Dr. Bianco.
The Fetal Cardiology team is like the hub of a wheel of services for both mom and baby. They helped coordinate Kelli’s transfer to Stanford Children’s, made sure her care appointments were seamless with maternal-fetal medicine, continued to monitor Hayden’s heart health, kept track of the care plan, and more. The program’s manager and care coordinator support families with all of their needs.
“As fetal cardiologists, we act as the baby’s primary cardiologist before birth, after birth, and throughout their life,” Dr. Kaplinski says. “Because we know the patient’s health history and care journey, we provide continuity for families and a friendly face throughout their care.”
On Oct. 30, 2020, Hayden came into the world, weighing 9 pounds, 6 ounces. To his parents’ delight, he arrived pink and breathing. A cardiac catheterization was performed shortly after birth to determine the size of his branch pulmonary arteries and MAPCAs.
Cardiac cath procedures and heart surgeries for improved heart/lung function
The cardiac catheterization—a procedure in which small, long, flexible tubes (catheters) are guided into the heart and blood vessels—measured blood flows and oxygen levels in Hayden’s heart and lungs. The test, performed in the Cardiac Catheterization lab, showed that Hayden’s blood vessels in his left lung were undeveloped.
“We took high-resolution pictures of all of the blood vessels going to the lungs, which helped us determine that Hayden needed surgery sooner than expected,” says Gregory Adamson, MD, an interventional cardiologist. “The images were then used to help create a 3-D roadmap for the surgical team to understand how to rebuild the pulmonary arteries the best.”
Learning that Hayden could not follow the typical surgical course for ToF—having one surgery between three and six months after birth—was challenging news for the family.
“About 85% of babies with ToF and pulmonary atresia don’t need surgery immediately,” Dr. Hanley says. Hayden was the exception. The safest path for him was a neonatal unifocalization and possible full repair (achieving all aspects of the unifocalization repairs at one time).
Hayden’s unifocalization surgery took place when he was just 19 days old. The surgery improved the blood flow to his lungs and jumpstarted the process of helping the blood vessels in his lungs to grow. However, Dr. Hanley could not complete the repair all in one surgery because of high blood pressure in Hayden’s lungs.
“During unifocalization, we start by building a new pulmonary artery using MAPCAs and reconstructing both lungs,” Dr. Hanley says. “Then, we test the pressure in the lungs mid-operation to see if we can continue. If pressures are normal, we complete the repair of the heart, something we can do 70% of the time.”
For Hayden, completing the repair, which includes closing the ventricular septal defect (hole) and placing a homograph conduit (prosthetic pulmonary valve) to connect the heart to the pulmonary arteries, would have to wait.
After his first surgery, he spent four months receiving state-of-the-art care in the Cardiovascular Intensive Care Unit (CVICU) growing stronger. The CVICU team are experts at supporting children with highly complex hearts with comprehensive, innovative care.
“It was a hard four months, but the teams were great. They looked at Hayden and saw the possibilities,” Kelli says. “There was a lot of brainstorming to find just the right solutions to his care needs. At the end of the day, they just wanted Hayden to have a healthy life, and they gave him that.”
At 9 months of age, Hayden returned for a cardiac catheterization to determine whether his unifocalized pulmonary arteries had developed enough for him to undergo the next stages of the cardiac repair. The result was positive, so he returned to the hospital to complete the surgery on his heart. This time, his hospital stay was brief. Through the course of his care, he had several more cardiac catheterizations, but the last one is most memorable.
“We performed a cardiac catheterization one year after Hayden’s surgeries, which revealed normal pressures in his lungs and a beautifully developed pulmonary artery system, confirming that the surgeries were successful,” Dr. Adamson says. “He’s now a happy and normally developing toddler, which is a testament to the excellent intensive care and rehabilitation services he received during his many months in our hospital.”
Home and enjoying an active, full life
Besides the great medical care Hayden received, Kelli is also grateful for the support services she received from Social Services and Child Life and Creative Arts. She says her social worker carried her through and advocated for her and that Child Life helped keep Hayden on track developmentally by providing extra therapies, including reading and music.
“It is a little bit of a miracle. It was a lot of talent, science, and faith all working together to keep him here,” Kelli says.
She revels in watching Hayden play and grow. Besides sports, he loves solving puzzles and being outside, but mostly he loves his family—his dedicated, loving mom; his best-friend dad; and his big sister, Hadley.
“Dan is the most amazing dad. He wants to make sure Hayden is always laughing and smiling. And Hayden loves Hadley, his sister. He’s always hanging out with her and her group of 11-year-old friends,” Kelli says.
While Hayden will need minor heart surgeries in the future to change out his conduit to larger ones as he grows, the toughest challenges are behind him.
“His quality and length of life are both markedly improved, and his blood flow is similar to yours and mine,” Dr. Hanley concludes.
