Advance with MUSC Health

Leading with the heart: The Advanced Fetal Care Center at MUSC Women's Health cares for families with prenatally diagnosed birth defects

Matthew Greseth
May 10, 2023
Dr. Eugene Chang with a patient
If light is in your heart, you will find your way home. - Rumi

Katie Williams and her husband were pleasantly surprised when they found out they were expecting their third child. Although her pregnancy was high risk, with complications like gestational diabetes and preeclampsia, they looked forward to welcoming Lucas to the world. And while everything had gone well during the beginning of her pregnancy, during a routine checkup, they learned some heartbreaking news. 

“On July 29, 2021, we went in for our 20-week scan, and the doctors found that Lucas had only three visible chambers in his heart,” said Williams. 

The Williamses were told that Lucas had hypoplastic left heart syndrome (HLHS), a rare congenital heart defect in which the left side of the heart is underdeveloped. The Centers for Disease Control and Prevention estimates that 1 out of every 3,841 babies born in the United States has HLHS. 

Normally, the left side of the heart is responsible for pumping oxygen-rich blood throughout the body; however, in patients with HLHS, the left side of the heart is weakened and cannot properly pump oxygenated blood to the body. 

“After we found out Lucas had HLHS, at that point, nothing went as planned,” recounted Williams. 

Planning for the unexpected

Babies born with congenital heart defects often need multiple surgeries to treat their heart problems. Williams and her doctors in Spartanburg decided it would be best for the family if MUSC Health handled Lucas’ care. 

Managing cases like HLHS is complex, and MUSC’s Advanced Fetal Care Center (AFCC) is experienced at coordinating care. The AFCC is the only fetal care center in South Carolina and receives referrals from across the region. The majority of patients who come to the AFCC have structural heart defects, like HLHS. MUSC also has the only pediatric cardiothoracic surgery program in South Carolina, which was ranked the No. 4 program in the country by U.S. News & World Report 2022-23. Last year, the AFCC served over 400 families, a 20% increase over 2021. 

A major goal of the AFCC is to maximize the prenatal diagnosis so as to determine a plan quickly for the family. Eugene Chang, M.D., an MUSC Health maternal fetal medicine specialist with a subspecialty in obstetrics and gynecology, and his team help to coordinate the 15 pediatric subspecialties that are required to care for medically complex cases such as structural heart defects.

“We meet as a multidisciplinary group to review the imaging, to review any previous genetic testing that may have been done and meet with the family as a group to discuss our findings,” explained Chang. 

Importantly, the multidisciplinary group guides the family throughout this process. By reviewing the case holistically, together they determine and execute a plan for the safest birth and best outcomes possible. 

Mending a broken heart

On a Sunday morning in mid-November, Williams stayed home from church. She had bad heartburn and didn’t feel up to leaving the house that morning. As the morning progressed, she continued to feel ill and eventually went to the hospital, which was a good call, as her blood pressure had skyrocketed, and she was going into labor. With Lucas’ heart condition, doctors needed to lower her blood pressure so she could be life-flighted from Spartanburg to MUSC. 

Katie made it safely to the MUSC Shawn Jenkins Children’s Hospital where doctors delivered Lucas and took him to the pediatric cardiac intensive care unit. While there, they gave Lucas medication to prevent his patent ductus arteriosus (PDA) from closing. The PDA is one of two openings that connect the left and right sides of the heart during fetal development. These openings normally close within a few days. 

But for patients with HLHS, these openings serve a critical function in the first few days after birth. They allow oxygenated blood to bypass the left side of the heart where it is then pumped to the rest of the body by the right side of the heart. While not a permanent solution, maintaining these openings allows for a sufficient supply of oxygenated blood to the body until the team can perform surgery, offering a more permanent solution. 

“At 5 days old, Lucas had his first open-heart surgery,” said Williams.

Lucas has now undergone three open-heart surgeries – the other two occurring when he was 4 weeks old and 5 months old. He will likely need one more surgery in the next few years. Ultimately, this series of surgeries reroutes blood flow in the heart, bypassing the poorly functioning left side of the heart and allowing the right ventricle, the lower chamber of the heart, to become the main pumping chamber. These complex surgeries will not cure Lucas’s HLHS but, rather, allow him to live with a single right ventricle heart. 

A bright future

small child crawling on floorWhile any diagnosis of a congenital heart defect is terrifying, the AFCC care team helps to alleviate some of that worry from families. The AFCC strongly emphasizes pediatric palliative care, which has a different focus than traditional palliative care. 

“There is a big focus on the family,” explained Chang. “We want to take care of the whole family; this is not just about the unborn baby but also the stresses that are on that family. They suddenly have an unanticipated diagnosis, which leads to transferring their care to a new obstetrician. That support from our palliative care team, as a family support team, is crucial to what we do.”

And the Williamses are extremely grateful for all of the support and care they received at MUSC. 

“It was exactly what our doctors in Spartanburg told us it would be like – but even better,” said Williams. “They looked at Lucas’ case and made decisions based on him. His care was completely tailored to him. I really did appreciate that they took the time to become Lucas experts, and we never had to worry about the decisions that were made for his health because they knew him so well.”
Although surgeons have treated Lucas’ heart defect, he is not cured. He will need regular follow-up visits with a cardiologist to monitor his heart condition, but his doctors said he should live a high-quality life. 

“Lucas is doing great,” said Williams. “He’s meeting every one of his milestones.”

Lucas is home with his family, and his future is indeed full of light.

Updated: 11/20/23