Our daughter's heart journey leads to advancements in subaortic stenosis research

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Photo courtesy of Lew and Laura Moorman

Like many young adults, 19-year-old Sarah Moorman has many things to look forward to. This fall, she began her freshman year at Duke University. While heading to college marks a big step for a teen as they transition from adolescence to adulthood, reaching this milestone is nothing short of a miracle for Sarah.

Lew and Laura describe their daughter as a courageous heart warrior. That description is befitting in so many ways. When Sarah was 9 months old, she had her first open heart surgery. Four years later, she had another open heart surgery followed by a third open heart procedure before her 10th birthday. The Moormans remember their daughter’s journey at Texas Children’s Heart Center® like it was yesterday.

“While this was a challenging journey for our family, our brave daughter was a trooper and overcame adversity at such a young age,” Laura said. “Today, it’s amazing to see her thrive and just be a normal teenager. She is so excited about her future, and we are proud of her and all that she has accomplished.”

The Moormans’ heart journey began when Sarah was 7 months old. She had gone to the pediatrician because she had a cold. When the pediatrician listened to Sarah’s heart, he could hear a murmur he thought was likely benign, but suggested Sarah see a cardiologist just to confirm.

“We scheduled an appointment with our pediatric cardiologist near our home in San Antonio,” Laura said. “As the doctor performed the echocardiogram, she noticed that Sarah had a large hole in her heart. She was diagnosed with a primum atrial septal defect (ASD) and her mitral valve was leaking. The only way to repair her heart was through open heart surgery. After consulting with a heart surgeon at Texas Children’s Hospital who had done hundreds of procedures on tiny patients, I was confident about having Sarah’s surgery performed there. I was impressed they had an entire floor dedicated to heart patients and the nurses were trained in cardiac care. The hospital near our home didn’t have a dedicated cardiac floor or staff.”

In April 2003, 9-month old Sarah had her first open heart surgery. After her surgery, she began seeing her cardiologist in San Antonio for regular check-ups. During one of those follow-up visits, her cardiologist noticed tissue growing on the subaortic membrane below the aortic valve.

“The growth was small at first,” Laura said, “but the cardiologist warned us that once it grew to a certain size, the subaortic membrane had to be removed or it would cause stenosis which obstructs the flow of blood to the left ventricle. When Sarah was 4 years old, the growth reached that certain size, and Sarah had her second open heart surgery at Texas Children’s to remove the subaortic membrane and also add a stitch to the mitral valve that had a minor leak. Then, at her next cardiology appointment, the subaortic membrane showed signs of growing back again. Eventually it started to grow up on the valve and keep it from functioning properly. Our daughter, now 9 years old, endured a third surgery to carefully remove membrane from the valve and to more aggressively treat the area to try to keep it from growing back.”

They had high hopes this would be her final surgery yet when the Moormans took Sarah in for a follow-up visit, her echocardiogram showed evidence of a membrane again. At this point, Laura and her husband were willing to hire a dedicated person to review all research that was conducted on subaortic membranes from around the world. They were desperate for answers to see what could be done to prevent this membrane from continuing to grow back. Was there a better procedure others were doing? What was causing the membrane to grow in the first place? They knew she couldn’t just continue to have open heart surgery every few years.

Laura and her husband turned to Sarah’s heart surgeon to help them find someone to do this research. He then enlisted the help of Dr. Sundeep Keswani to oversee subaortic membrane research at Texas Children’s and Baylor College of Medicine. From that moment, the research wheels were set in motion. 

“We learned there was very little research out there on subaortic membranes, so I felt somewhat relieved that we weren’t missing out on anything obvious,” Laura said. “Apparently, nothing had been done to explain why 30 percent of patients have a more aggressive form of disease where these membranes grow back. There are theories but no proof. The only current treatment was open heart surgery every time.”

With support from the Moormans, Dr. Keswani and his team at Texas Children’s and Baylor College of Medicine – in collaboration with scientists at Rice University – obtained a National Institutes of Health grant to study the dynamic processes and cellular players linked to discrete subaortic stenosis (DSS).

The researchers developed computer and tissue-engineered models to predict the recurrence of DSS lesions of the left ventricular outflow tract. In normal hearts, blood passes through the tract from the left ventricle to the aorta. In patients with DSS, like the Moormans’ daughter, a fibrous tissue forms and prevents blood flow from leaving the heart. 

“When we examined the subaortic membranes that were resectioned from patients including Sarah’s, we found striking differences between those who had the aggressive form of the disease and those who did not,” said Dr. Keswani, chief of pediatric surgery at Texas Children’s. “When a skin injury occurs, it sets off a cellular cascade of events that can lead to fibrosis, and that is what’s happening with this disease. Since we now have genomic data, we can determine which patients are more predisposed to injury and more likely to see a fibrous re-growth. Our findings are giving us insight into the mechanism of how this membrane forms and why some humans have a more pro-fibrotic phenotype, which will help us change the way we manage this disease so we can improve the quality of life for these children. Along with the progress and better understanding we’ve made in this area, it is important to note that our work would not be possible without the support of our surgical leadership team, our NIH and Rice partnerships, and the philanthropic support from the Moormans, who have been staunch advocates from the very beginning.”

For the Moormans, it’s not just about doing the research. It’s about collaboratively sharing this research and data with others to help the next generation of families. The hope is to improve treatment in children with DSS and those children with other congenital heart conditions that share similar disease processes.

“I am grateful to Dr. Keswani and his team for advancing subaortic membrane research,” Laura said. “Miraculously, since this research project began, Sarah’s subaortic membrane stopped growing. Her cardiologist said it looks exactly the same as it did nine years ago. Our daughter is doing amazingly well, is on no medications and has no physical restrictions. I did extensive research of other children’s heart hospitals and spoke with parents of other heart patients at each. I’m glad I chose Texas Children’s for Sarah’s care.  As a hospital focused on subaortic membrane research, I know we are in a better place now to create life-changing progress.”

Click here to learn more about Texas Children’s Heart Center. Click here to learn more about the subaortic stenosis research at Texas Children’s, Baylor College of Medicine and Rice University.