Healing a tiny heart: baby Felix’s journey with Truncus Arteriosus

When Sarah and Johnny Leo learned they were expecting their first child in early 2023, the couple was overjoyed at the prospect of welcoming a baby into their young family. But during her routine anatomy scan at 18 weeks’ gestation, Sarah’s OB-GYN discovered that there was something wrong with the structure of their baby’s heart. Instead of having two main vessels come out of the heart—the main pulmonary artery and the aorta—their tiny baby only had one, a rare defect known as Truncus Arteriosus.  

The couple was devastated by the news, and especially the uncertainty that came with it.  

“We cried for days and kept wondering what else might be wrong,” Sarah recalled.  

That same week, Sarah met with Shannon Powell, MD a cardiologist at the Heart Center at Children’s Hospital New Orleans. Dr. Powell performed another comprehensive anatomy scan, as well as an echocardiogram on her baby’s tiny heart, which at that point was just the size of an olive.  

The echocardiogram, which uses ultrasound imaging to provide a clearer picture of the structure and function of a heart, confirmed the diagnosis of Truncus Arteriosus—while also removing anxiety about additional complications.  

“Dr. Powell explained that it was a complex condition with a relatively ‘easy’ fix,” Sarah recalled. “She definitely made us feel better; she gave us answers and reassured us that there was a solution.”  

The solution would be a highly specialized surgery that would be performed after their baby was born. For the remainder of her pregnancy, Sarah continued to see Dr. Powell regularly for ultrasounds and echocardiograms. Despite her baby’s congenital heart condition, her pregnancy proceeded normally with no additional complications.  

“The goal was for me to go full term, because the more developed my baby was, the safer it would be to operate on him after he was born,” Sarah said. “He ended up coming at 39 weeks and everyone was happy that he was so big—it was definitely a bright spot during such an anxious time.”  

Felix Leo was born at a local hospital on Friday, December 15, 2023, weighing 7 lbs., 11 oz. Originally, Sarah and Johnny were prepared for him to be transferred immediately to the CICU at Children’s Hospital New Orleans, but because he was so big and otherwise healthy, they were able to spend some time in the room with him immediately after his birth, with the specialists at the local hospital on standby.  

Later that evening, Felix was transported by ambulance to Children’s Hospital New Orleans and admitted to their Cardiac ICU. His surgery was scheduled for the following Monday morning and was performed by Frank Pigula, MD, Chief of Pediatric Cardiothoracic Surgery and Co-Director of The Heart Center at Children’s Hospital New Orleans.   

“For Truncus Arteriosus, we use a patch to close the ventricular septal defect, which is the hole in the heart between the left and right ventricles,” Dr. Pigula explained. “We then create a new aorta using the existing single blood vessel that is there, and we integrate an artificial conduit and valve into the structure, creating the two valves needed for proper function.”  

On the day of the surgery, Sarah and Johnny were anxious but ready. Sarah recalled being thankful that Children’s Hospital New Orleans used a software called EASE (Electronic Access to Surgical Events) App, which allowed nurses and staff inside the operating room to provide regular updates to them.    

“It was a relief to get that constant communication, and we were able to keep our family updated easily that way, too,” Sarah said.  

The surgery was estimated to take approximately five to six hours, and at six hours on the dot, Dr. Pigula came into the waiting room to let Sarah and Johnny know that everything had gone well.  

“We were just so relieved,” Sarah said. “The months of mental preparation leading up to this point were as challenging as the surgery itself.”  

Felix remained in the hospital for approximately three weeks recovering—another happy surprise, since it was originally estimated to be as long as six weeks.  

“It was a rollercoaster at first, with his heart stopping the night after the surgery, but Dr. Pigula came back that evening and made sure everything looked good,” Sarah said. “They spent a lot of time working to get his fluid and blood pressure under control, but over the next week he got better so quickly—it was really amazing to watch.”  

Sarah, who actually worked as an Allergy and Immunology Fellow at Children’s Hospital New Orleans at the time of Felix’s surgery, said that in addition to the amazing medical care Felix received, she was impressed with her experience as a family member of a patient—a role reversal she could never have imagined.   

“I remember so many of his nurses, who were supposed to work three days in a row, but they would pick up more nights so that they could work with him and ensure continuity of care,” Sarah said. “The nurses just never stopped moving the entire time they were in the room—they were just up and down checking drains, medications, moving things around—just back and forth from the computer to him 12 hours straight. I honestly don’t know how they do it, and it was amazing to watch.”  

As a physician who also completed her residency and chief year at Children’s Hospital, Sarah said she has a newfound appreciation for the organization she already admires and loves.  

"My experience as a patient was unique, having worked at the hospital for six years,” Sarah said. “The support was remarkable, especially the unexpected care from the Maternal-Fetal Medicine nurse, as well as Child Life, who had a gift drive for patients who were in the hospital over the holidays. The frequent visits from lactation consultants were a huge help, and the respiratory therapists were incredible too, always communicating effectively and advocating for us during rounds. The entire support staff's dedication was truly amazing." 

Sarah and Johnny’s extended family were able to come visit during the hospitalization, which fell over the Christmas holiday, and the nurses even found a “Baby’s First Christmas” hat for Felix.  

“It was way too big for him but so cute,” Sarah said. “Obviously we never would have wished to be in the hospital over the holidays, but if you have to, Children’s Hospital was a special place to be.”  

Sarah also said she and Johnny loved the Beads of Courage program they participated in while Felix was in the CICU.  

“Every day, we'd fill out a checklist of different obstacles and milestones that Felix overcame which were reflected by various beads,” Sarah recalled. “Scary obstacles like long nights in the hospital, code events, and open-heart surgeries are there, followed by milestones like staple removals, his first bath, and coming off oxygen. It helped us celebrate the little steps forward during the long days when progress can seem so slow. Nothing made us more excited than putting on the little silver house before we took him home!” 

After his discharge home, however, he had an echocardiogram during his 1-week follow up visit that revealed a growth on his truncal valve, which after the initial repair was analogous to the aortic valve between the left ventricle and the aorta.  

“He was readmitted to the hospital that night and after a few tests, it came back that he had an infection,” Sarah recalled. “He was immediately started on antibiotics, and then Dr. Pigula performed another open-heart surgery a few days later to replace that valve.” 

Felix recovered at Children’s Hospital New Orleans for another three weeks and has been home since, growing bigger and stronger by the day.  

“The second surgery was more urgent, but the post-op felt smoother—maybe because we’d gone through it all before,” Sarah said. “We still go in for follow up appointments, and Felix loves when his therapists come to our house to help ensure he’s on track with his development after his two major heart surgeries.”  

Today, Felix is teething, rolling over, and his scar has almost completely faded.   

“We are just so thankful,” Sarah said. “Everyone was as prepared as they could be for the unexpected infection, and I was thankful that the doctors were always so honest with us, which can be a hard thing to do, especially when the outcome is uncertain. We are truly grateful for the amazing care we received and the priceless gift of our baby’s health.”  

To learn more about the Heart Center at Children’s Hospital New Orleans, visit https://www.chnola.org/services/heart-center/