Early detection of rare heart defect leads to lifesaving operation in the minutes after birth
Violet Wright was 20-weeks pregnant with her first child, Atticus, when a routine ultrasound led to an alarming discovery: the left side of Atticus’ heart wasn’t visible on the scan.
As a midwife, Violet feared the worst – a rare and life-threatening congenital heart defect called hypoplastic left heart syndrome (HLHS), which happens when the heart’s left side doesn’t develop correctly. She met the next day with Jessica Nyholm, MD, a maternal-fetal medicine physician with M Health Fairview and an associate professor in the Division of Maternal-Fetal Medicine at University of Minnesota Medical School. While Nyholm – who specializes in high-risk pregnancies and deliveries – confirmed that Atticus likely had HLHS, she also gave the Wrights hope.
“She delivered the news very compassionately,” said Violet. “Even though it was a difficult day, we were glad we were able to work with her and that she started walking us through this journey.”
Nyholm connected the family with our fetal cardiology team at M Health Fairview Masonic Children’s Hospital, and they met the next day with Kavisha Shah, MD, a fetal cardiologist at our children’s hospital and faculty member in the Division of Pediatric Cardiology at University of Minnesota Medical School.
Shah performed a fetal echocardiogram – an ultrasound test designed specifically to evaluate a fetus’ heart – and was able to take a closer look. The only issue at the time was what the ultrasound had suggested: the left side of Atticus’ heart wasn’t developing, but future monitoring would paint a more complicated picture as he continued to grow in the womb.
What is hypoplastic left heart syndrome?
Children with HLHS are essentially born with only one functioning side of the heart. In a standard heart, the right ventricle pumps oxygen-poor blood to the lungs to become oxygenated; the left heart then takes the oxygenated blood from the lungs and pumps it to the rest of the body.
In Atticus’ case, his left ventricle couldn’t pump blood to his body, meaning his right ventricle had to take on both tasks. For this to be sustainable, the right ventricle needs to be healthy, and there should be adequate mixing of blood between the right and left upper chambers of the heart.
Lack of connection complicated Atticus’ outlook
Shah, who is part of our dedicated single-ventricle care team, continued meeting the family and performed a series of routine fetal echocardiograms leading up to Atticus’ birth.
When Violet was 30-weeks pregnant, a scan revealed that the opening between the two top chambers of Atticus’ heart had completely closed. Coupled with the fact that his left lower chamber was small and wasn’t developed properly, this made his situation even more serious.
Doctors would have to work quickly in the minutes following his birth to create a hole in his atrial septum, so that oxygenated blood could flow from the left upper to right upper chamber and circulate to the rest of his body. The intact atrial septum also posed a risk to him in the womb because it could cause his lungs to develop poorly. Shah ordered a fetal MRI to get a more in-depth picture and saw that, thankfully, Atticus’ lungs were forming well.
