Creating New Paths

The first four months of Yaheng Lu’s pregnancy were routine. But when Yaheng and her husband, Jeff, went in for her 20-week ultrasound, her OB-GYN and ultrasound technician noticed something unusual, and the appointment took a different tone.  

Yaheng’s OB-GYN referred her to Fetal Heart Center, a partnership between St. Louis Children’s Hospital, WashU Medicine, and Barnes-Jewish Hospital, for a fetal echocardiogram. The scan revealed that the baby had two rare congenital heart conditions—truncus arteriosus and an interrupted aortic arch—that would require surgery right after birth. Left untreated, these diseases can lead to developmental, respiratory, and other life-threatening complications. The revelation left the first-time parents reeling.

“It was quite shocking and just took a long time for us to process,” Yaheng, 36, recalled. “I think we’re still processing it or, at least, I am.”

As sudden as the diagnosis was, Yaheng’s WashU Medicine pediatric cardiology providers got to work. By coordinating with her OB-GYN and taking a shared decision-making approach, the team created a proactive pregnancy care plan, leading to a smooth delivery, successful surgery, and a healthy family of three.

Some much-needed guidance

Truncus arteriosus occurs when a fetus only has one major artery exiting their heart—the aorta or pulmonary trunk—instead of both. In a healthy heart, the aorta pumps blood to the body and the pulmonary trunk directs blood to the lungs. Without both arteries, blood gets mixed and the heart gets strained from working harder to pump blood to the lungs. Yaheng and Jeff’s baby was also diagnosed with an interrupted aortic arch, which is an underdeveloped aorta that prevents blood from flowing as it should through the body.  

Approximately 1 in 16,000 babies have truncus arteriosus, while an interrupted aortic arch happens in about 1 in every 50,000 births—having both is extremely rare.  

The ultrasound sparked lots of questions for Yaheng and Jeff. In the short-term, they wondered what this would mean for the remainder of Yaheng’s pregnancy. And long-term, they worried what it would mean for both the baby and mother’s health.  

“We certainly didn’t know the severity because we’re not cardiologists,” Jeff says. “But there were a lot of factors to consider about Yaheng’s health and the baby’s health.”

Within days of the ultrasound, Yaheng and Jeff met with Caroline K. Lee, MD, a WashU Medicine pediatric cardiologist at Fetal Care Center to develop a birth plan.  

She referred Yaheng and Jeff to Pirooz Eghtesady, MD, PhD, a WashU Medicine pediatric cardiothoracic surgeon at St. Louis Children’s, to perform the surgery. St. Louis Children’s nationally recognized heart care provides family-centered treatments to patients like Yaheng, combining extensive pediatric cardiology knowledge with compassion to provide each patient with the exceptional care they deserve.  

Congenital heart defects are problems in the way the heart develops, and they occur in about 1% of newborn babies. A quarter of these babies require heart intervention in the first year of life.  

“By diagnosing heart defects before a baby is born, we can help prepare families about what to expect, what procedures will be needed, and can help coordinate OB-GYN and pediatric care for safe delivery for mom and baby,” Dr. Lee says.

Dr. Eghtesady explained what the surgery would involve, while Dr. Lee outlined the baby’s month-long recovery timeline and connected them to congenital heart disease parent support groups.    

“I really appreciated that Dr. Lee encouraged us to advocate for ourselves,” Yaheng says. “To me, that’s important in forming a long-term, trusting relationship with a health care provider.”

Ready for what’s ahead

In July 2023, Yaheng delivered a baby boy named Ben at Barnes-Jewish Hospital. Before his surgery, Ben stayed in St. Louis Children’s Level IV newborn intensive care unit (NICU), which provides critically ill babies the highest-possible level of care.  

Five days later, Dr. Eghtesady performed a 7-hour surgery on Ben at St. Louis Children’s and WashU Medicine Heart Center. The procedure included sewing together a pulmonary trunk for Ben, which he was born without, fixing the blood vessels to his lungs, and making repairs throughout the heart to improve the blood flow to other parts of Ben’s body.

After the surgery, Ben recovered at St. Louis Children’s. The 110-foot bridge connecting St. Louis Children’s to Barnes-Jewish enables parents to visit their newborns and bond while moms and babies get the extra attention they need.

“Maternal-infant bonding is so important for both mother and baby, and our teams try to ensure this can occur safely, even for a baby with a serious heart defect,” Dr. Lee says. “Time for holding the baby and skin-to-skin time in the delivery room enhance the connection between mother and newborn and can decrease maternal stress, so being close to where their baby is receiving care allows mothers to be at their baby's bedside from the start.”

Seventeen days after his surgery, Ben got to go home. These days, Ben is a healthy 2-year-old boy. He’s rambunctious, smart, and loves anything with a motor, according to his dad. Over the summer, he went on his first plane trip when his parents took him to Niagara Falls.  

Last April, he had a cardiac catheter implanted into a pulmonary valve to help improve blood flow from the heart to the lungs.  

Ben still sees Dr. Lee for his checkups, and his parents think he’ll need to have another surgery in a few years as he outgrows the pulmonary trunk he received as a newborn. And because children born with congenital heart defects face increased risk of developmental delays, Yaheng and Jeff take Ben for follow-up visits at the Cardiac Neurodevelopmental Clinic at St. Louis Children’s, which they believe is crucial to his overall quality of life in addition to his cardiac health.

“By collaborating with a multidisciplinary team of specialists, we’re confident that we’re doing the utmost to ensure the best possible quality of life for Ben, and we know this is a shared goal for many other families with kids with congenital heart issues,” Yaheng says.  

Yaheng and Jeff are thankful for the continuity of care at St. Louis Children’s and the compassion of the entire BJC HealthCare system.  

“We’d like to extend our most sincere gratitude to the doctors, nurses, therapists, Child Life specialists, dietitians, and social workers who play important roles and make our medical visits and stays much easier,” Jeff says. “Their support has been invaluable in helping our family navigate life with essential medical needs.”

The St. Louis Children’s and WashU Medicine Heart Center work to ensure your little one’s heart is as strong and healthy as it can be. From surgery to imaging to noninvasive procedures, our experts provide exceptional, nationally recognized pediatric heart health care. Learn more about the Heart Center.