What is Hypoplastic Left Heart Syndrome?

Hypoplastic left heart syndrome is a rare congenital heart defect that occurs when one part of the heart (left ventricle) does not develop completely.

“It isn’t just that the ventricle, or pumping chamber, is small. It isn’t being used properly, so it’s not developing, and it stays small, or hypoplastic,” explains William Polzin MD, director of Maternal-Fetal Medicine at Good Samaritan Hospital and Co-Director of the Fetal Care Center of Cincinnati.

Diagnosing Hypoplastic Left Heart Syndrome

This condition can be detected by an ultrasound if the ultrasound quality is high enough. However, hypoplastic left heart syndrome, as well as other congenital heart defects, is frequently missed because of ultrasound quality.

For this reason, the American Institute of Ultrasound and Medicine (AIUM) is pushing to more carefully credential centers that are doing obstetric ultrasound. “The AIUM has certain standards, based on volume of ultrasounds done and certification of the people reading them, which makes these conditions more likely to be picked up,” Dr. Polzin points out. “The Seton Center at Good Samaritan Hospital is credentialed and certified by the American Institute of Ultrasound and Medicine (AIUM)."

If your baby has this condition and it is not detected before birth, he or she would be diagnosed very shortly after birth, by your nurse or pediatrician, due to rapid deterioration of health. Newborns with hypoplastic left heart syndrome may present the following symptoms within the first few hours of life:

  • Bluish or poor skin color
  • Cold hands and feet
  • Lethargy
  • Poor pulse
  • Poor sucking and feeding
  • Pounding heart
  • Rapid breathing
  • Shortness of breath

How is Hypoplastic Left Heart Syndrome Treated?

Once your baby’s diagnosis is confirmed, usually by an ultrasound called echocardiography, he or she will be evaluated more fully and set up for immediate post-natal therapy. This will be done in the neonatal intensive care unit (NICU) if diagnosed before birth. Your baby's care team will determine when he or she needs to be treated, and with what type of procedure. From there, your baby may need to be put on a breathing machine with oxygen, and they may be given a medicine called prostaglandin E1 to keep blood circulating to the body.

“Sometimes the heart is developed enough to allow it to function independently, so the baby would retain function of all four heart chambers, but only rarely” Dr. Polzin explains.

Treatment almost always require a series of operations – usually three – to reconfigure the heart, resulting in one ventricle (the right ventricle) doing the work of both.

How Will This Condition Impact My Child Later in Life?

Your child will most likely need to take daily medications and will need close following by a cardiologist throughout his or her life. “It depends on the severity,” Dr. Polzin explains. “Typically, they will not be able to be athletes of any kind, but they can be very functional in the community – playing with friends and general fitness exercise.”

If the condition progresses or worsens, some children require a heart transplant. “Then, that’s a life-long situation. While the heart may be working better, the medication and therapy that is chronically required for transplant patients causes some limitations in health,” he adds.

Tags Heart