Lifesaving Surgical Care for Patient with HLHS

Patient Stories

Aiden Rodgers HLHS 0

Aiden Rodgers is a fun-loving and mischievous 2-year-old who has no idea how serious his early days were. But his parents know that if it weren’t for the close teamwork between Texas Children’s Fetal Center and Heart Center, he might not be here today.

When his mother, Jenny, was 20 weeks pregnant, she learned the fetus she was carrying had hypoplastic left heart syndrome (HLHS), a hole in the wall separating the upper chambers of the heart. She was told he would need at least three open-heart surgeries, the first just days after his birth, and even then his chances of survival weren’t certain.

“It was probably the worst day of our lives,” she said. “The weeks that followed were a blur of tears, prayers, desperation and grieving the loss of the life we had envisioned as a family.”

After the diagnosis, she and her husband got to work researching fetal surgical centers. They found one of the best in Houston, just three hours away from their home in Louisiana.

At their first visit to the Fetal Center, a coordinator set up appointments with their personal team of experts and guided them through the process. They met with Nancy Ayres, MD, director of non-invasive imaging and fetal cardiology, who spent hours checking Aiden’s heart with fetal echocardiograms. They also met with obstetrician, Richard Ivey, MD, toured the CVICU and met Jeffrey Heinle, MD, associate chief of congenital heart surgery, who would operate on their newborn’s heart.

“To be able to do all of this prior to having Aiden was just invaluable,” Jenny said. “Having everyone involved in Aiden’s care under one very large roof was a great comfort and a huge convenience.”

When Aiden was four days old, he had his first surgery, a Norwood procedure. After six weeks, he was discharged until his next surgery, a Glenn procedure at 4 months old. After that major operation, Aiden and Jenny had to remain in Houston for two weeks before going home.

“To say it was a joyful homecoming is an understatement,” Jenny said. “To have Aiden and his older brother home together was the most amazing thing.”

Now, when Aiden has periodic checkups, his appointments are scheduled with the entire team all in one day to make it easier on the family.

“Aiden still has at least one more surgery to face, and his heart will never be completely fixed,” Jenny said. “He will always have half of a functioning heart, but with the advancements in medicine, we pray he will have a long and wonderful life.”

The family is grateful for Texas Children’s expertise and the incredible life Aiden has been able to lead thus far.

“It helps to have so much love and care surrounding you when you’re dealing with a child with a very complex heart,” Jenny said. “This whole journey is not easy – not one second of it. But we have hope because of Texas Children’s Hospital.”

Fetal Cardiac Interventions

Hypoplastic Left Heart Syndrome and Associated Conditions

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect that occurs when the left side of the heart does not form properly. The mitral valve, the left ventricle and the aortic valve are too small, and there is not enough blood pumping through the left side of the heart to support the rest of the body. Related conditions include evolving HLHS, where the valve is beginning to narrow and there is concern that the baby will have HLHS by the time of birth, and borderline left heart, where multiple left-sided structures are smaller than they should be. Texas Children’s Hospital has successfully performed in-utero fetal cardiac interventions for all three conditions. With a coordinated effort among a large, multidisciplinary team of fetal cardiologists, obstetricians and gynecologists, interventional cardiologists, congenital heart surgeons, fetal imaging experts, maternal and fetal anesthesiologists and other clinical specialists, Texas Children’s Hospital is the first in the southwestern U.S. to create a program to treat these defects in utero.

Catheter-Based Fetal Aortic Valvuloplasty for Fetal Aortic Stenosis (Evolving HLHS)

Texas Children’s Hospital has successfully performed this catheter-based procedure for babies with severe aortic stenosis in utero (evolving HLHS). A tiny balloon is inflated inside the aortic valve to open up the leaflets, after which the balloon is then removed. This allows blood to flow more easily through the left side of the fetal heart and potentially improve growth of left-sided structures.

Catheter-Based Atrial Septal Interventions for HLHS with Intact or Restrictive Atrial Septum (RAS)

Babies who already have HLHS in utero with no potential for growth of the left-sided structure depend on a hole in the atrial septum to keep blood circulating throughout their body. In some babies, this hole either does not exist or it is too small, and they have a high risk of complications and death. In a catheter-based procedure, we use a balloon or stent to create an atrial septal defect (a hole between the top chambers of the heart) to keep blood flowing through the fetal heart.

Chronic Hyperoxygenation for Borderline Left Heart

For fetuses with borderline left heart structures, Texas Children’s Fetal Center was the first in the country to offer a research protocol in which mothers receive daily oxygen therapy throughout their third trimester. By delivering extra oxygen to the mother through face mask or cannula, we hope to increase the amount of oxygen in her blood, the amount of oxygen going to the placenta and fetus, and ultimately the amount of oxygen flowing into the fetal lungs and into the left side of the baby’s heart. By improving flow to the left side of the heart, growth should improve as well. We are also studying the effects of extra oxygen for the fetal brain. We are currently enrolling mothers in the first randomized controlled trial of this therapy.

Learn more about Hypoplastic Left Heart Syndrome

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