Skylynn’s story: A first-of-its-kind heart procedure at Children’s Hospital New Orleans

Skylynn Welch is an 8-year-old active second grader who loves sports and the outdoors. She is involved in many competitive sports – like basketball, softball, cheer, and gymnastics.

But Skylynn had to put these activities on hold. In April 2023, she became the first pediatric heart patient at Children’s Hospital New Orleans to undergo a relatively new surgical technique, called the Ozaki procedure, to replace her bicuspid aortic valve, an aortic valve with two leaflets instead of three. Unlike mechanical valve replacement surgery, the Ozaki procedure used Skylynn’s own pericardium, tissue from the heart sac, to construct three leaflets for her new aortic heart valve.

The Ozaki procedure is the first-of-its-kind for the Gulf South, and Children’s Hospital is among a handful of centers in the nation offering this novel approach for patients with aortic valve disease.

“Skylynn is doing remarkably well after Ozaki surgery,” said Skylynn’s mom, Cynthia. “She’s begging me to let her do cartwheels, but I keep telling her it’s still too early to do that. Looking back on everything, I never imagined we’d be on this journey. Skylynn’s heart was fine until she was a few months old.”

Skylynn’s heart journey

When Skylynn was around 3 months old, Cynthia took her daughter to the pediatrician in Alexandria for a check-up. During that appointment, the pediatrician detected a heart murmur and sent her for further testing. At 6 months old, Skylynn had her first cardiology appointment in Alexandria with Dr. Anita Go, a pediatric cardiologist affiliated with Children’s Hospital New Orleans. After an EKG and echocardiogram, Dr. Go concluded that Skylynn had a serious aortic valve condition.

Skylynn had a bicuspid aortic valve which led to more heart complications including stenosis, the narrowing of the aortic valve causing blood flow to be reduced or blocked from the heart to the body, and aortic regurgitation, a condition that occurs when the heart’s aortic valve doesn’t close tightly. As a result, some of the blood pumped out of the heart’s left ventricle, the heart’s main pumping chamber, leaks backward, limiting blood flow to the rest of the body. Over time, Skylynn developed dilated cardiomyopathy, where the heart’s left ventricle becomes dilated or enlarged.

“As a mom, it was hard knowing my baby was very sick,” said Cynthia. “But I knew I had to put it in God’s hands that he was going to take care of her. We knew Skylynn would need surgery at some point in her life, but right now it was a wait-and-see process. We had regular follow-ups with Dr. Go to monitor any changes in Skylynn’s heart condition and address them as they happened.”

Over time, Skylynn’s aortic valve began to leak. She was given medicine to slow the progression of that leakage. Her heart valve condition seemed to be stable until she came in for her cardiology follow-up visit in February 2023. Dr. Go ran another heart echocardiogram and EKG on Skylynn.

“During that appointment, Dr. Go said Skylynn’s body was not growing at the rate of her heart and it was probably time for us to consider heart surgery,” said Cynthia. “Along with that, we learned Skylynn’s aortic valve leakage was getting significantly worse and her left ventricle was dilated.”

Dr. Go presented Skylynn’s case to the Children’s Hospital’s Heart Center Case Management Conference and they reached a unanimous consensus recommendation for surgical intervention.

“Two weeks later, I got a call from Dr. Pigula’s nurse practitioner, Jill LeBlanc, who told me that they reviewed my daughter’s case, and she would be a good candidate for a valve repair or valve replacement surgery. They wanted us to come down and meet Dr. Pigula to discuss it further.”

Discussing our surgical options

On March 10, 2023, Cynthia and her family met Dr. Frank Pigula, Chief of Pediatric Cardiothoracic Surgery and Co-Director of The Heart Center at Children’s Hospital, to discuss surgical options and to schedule Skylynn’s heart surgery.

“Dr. Pigula answered all of our questions and concerns,” said Cynthia. “He told us repairing the aortic valve would not be feasible because her aorta began to stretch. If the repair wasn’t going to work, we opted to move forward with the mechanical valve replacement surgery even though that meant she would be on lifelong blood thinners which would limit her physical activities. But two days before surgery, Dr. Pigula wanted to meet with us to discuss another option for surgery.”

Dr. Pigula introduced the family to a relatively new surgical technique called the Ozaki procedure, developed by Dr. Shigeo Ozaki in 2007. It is an innovative technique for aortic valve replacement that removes the diseased aortic valve leaflets and uses a patient’s own heart sac tissue to create three leaflets for a new aortic valve that expands as the heart grows, possibly extending the life of the repair. Unlike mechanical valve replacement, Ozaki procedure does not require patients to take lifelong blood thinners, since their blood does not adhere to the valve or circulate in a manner that leads to blood clots, which can be a concern with artificial heart valves.

“All this time, Dr. Pigula had been working behind the scenes to find the best surgical option for Skylynn,” said Cynthia. “He had been on the phone with many different pediatric surgeons across the U.S. who had performed the surgery, including the heart surgeon in Japan who created this procedure. He wanted to ensure Skylynn was eligible for this surgery before offering it to us.”

After weighing the pros and cons of the Ozaki procedure, and discussing it with her family, and praying about it, Cynthia and Skylynn’s father were more comfortable moving forward with it.”

Skylynn’s heart surgery

On April 7, 2023, Dr. Pigula and his team at Children’s Hospital performed the Ozaki aortic valve replacement procedure on Skylynn. The surgery lasted two hours and everything went as planned.

“I was a little nervous when she was wheeled into surgery, like any mom would be, but when Dr. Pigula told us – she did perfect – it was a huge relief off our shoulders,” said Cynthia. “When she woke up, she was in pain, but she was more excited to see her 8-inch warrior scar on her chest.”

She said, “Mommy, it’s done. I have my miracle heart.”

“We were impressed with Dr. Pigula,” said Cynthia. “He did an outstanding job, and we appreciated all his time, effort, and research in helping us determine the best treatment approach for Skylynn.”

Dr. Pigula says the Ozaki procedure is still a fairly new technique in children, but he and his team at the Heart Center believe it holds great potential in the field of pediatric cardiovascular surgery.

“While the Ozaki technique has been used in adults with aortic valve dysfunction for over a decade with promising long-term outcomes, we anticipate similar results in our pediatric population,” said Dr. Pigula. “The Ozaki procedure, which is not widely available in pediatric hospitals, is particularly advantageous for children. The newly created valve has the potential to accommodate the heart’s growth and continue functioning effectively as a valve. By offering this new option at Children’s Hospital, we are revolutionizing the care we provide for patients with aortic valve disease who may not be suitable candidates for traditional valve replacement surgery. With the Ozaki technique, Skylynn will not require lifelong anticoagulant therapy, and she can live a normal, active lifestyle.”

The first few days of Skylynn’s recovery were difficult, but she recovered relatively quickly and was discharged from the Cardiac Intensive Care Unit (CICU) six days later. She’ll be on blood thinners for the first three months and then will take one aspirin a day. She also sees Dr. Go for regular follow-up visits.

“We had a wonderful experience at Children’s Hospital,” said Cynthia. “We were impressed with all the doctors, nurses, and staff who treated Skylynn with one hundred percent care. Skylynn’s middle name is Faith. She has shown a lot of faith through this process. Faith that God was going to provide for what she needed and give her strength to get through it, and faith that God was going to protect the surgeon’s hands. Our miracle came right at the last minute. If it wasn’t for Dr. Pigula, Skylynn wouldn’t have been offered this new surgery, and he wouldn’t have had the privilege to do this surgery on her, and possibly offer it to other kids. Our journey was stressful but if it opens the door for other kids to live a normal life, it was all worth the stress and worry that came with it.”

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