Story by: Patrick Donovan and Lynne Choate on November 23, 2018
One of the rarest and most complicated heart defects found in babies is hypoplastic left heart syndrome (HLHS) in which a critical underdevelopment of the left side of the heart leaves the right side doing most of the work.
“With hypoplastic left heart syndrome, the left side of the heart can’t effectively pump blood to the body,” said Joshua Sparks, M.D., medical director of the Pediatric Advanced Heart Failure and Transplant Program team at Norton Children’s Hospital and pediatric cardiologist with UofL Physicians.
The result for infants with HLHS is that oxygen-rich and oxygen-poor blood mix in their heart. They experience trouble breathing and rapid heartbeat. Because of poor circulation, their extremities are cold and the skin, lips and nail beds take on a blue color.
It’s widely unknown why defects such as HLHS occur, but suspected causes include genetics, certain medications taken during pregnancy, drug and alcohol use while pregnant and viral infections.
Although HLHS treatment isn’t exactly the same for each child, many undergo a series of surgical procedures. The timetable for these procedures can vary based on growth, overall wellness and organ development. The surgical procedures include:
Learn more about Norton Children’s Heart Institute, affiliated with the University of Louisville, or schedule an appointment.
These surgeries don’t cure HLHS. The child will need regular follow-up visits with a pediatric cardiologist to monitor progress. When the child becomes an adult, a congenital heart defect specialist will continue to provide care.
In some cases, if the HLHS is very complex or the heart becomes weak after any of the surgeries, a heart transplant may be needed.