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Hypoplastic left heart syndrome (HLHS) is an abnormality of the heart that a baby is born with. The left side of the heart is smaller and weaker than it should be. HLHS surgery is required to create new pathways for the heart to pump oxygen-rich blood through the body.
Since the left side of the newborn’s heart doesn’t pump enough blood, the right side of the heart has to work harder but won’t be getting enough oxygen from the lungs.
A baby born with HLHS often has an atrial septal defect (ASD) — a hole between the ventricles that actually helps their heart function. The hole allows oxygenated blood from the left ventricle mix with the blood being pumped out to their body.
Babies born with HLHS but without this hole will need an emergency intervention called a balloon septostomy shortly after birth to create such a hole.
Another hole — the patent ductus arteriosus (PDA) — is normal and usually closes within the first few days of life. In a baby with HLHS, this hole also helps mix the oxygenated blood and needs to be kept open. Prostaglandin is a medication frequently given to babies with HLHS after birth to keep the PDA open. The cardiothoracic surgeons at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, are prepared and experienced to act swiftly to keep the PDA and the ASD open until the heart is repaired with HLHS surgery.
It wasn’t long ago that babies born with HLHS didn’t survive. Using the latest techniques, the board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to effectively rebuild a baby’s circulatory system with HLHS surgery, helping them to potentially live a full and productive life.
HLHS may be seen on ultrasound scans during pregnancy. A fetal echocardiogram provides more information and tells the delivery team how to prepare for care after birth.
Common tests used to learn more about a newborn’s heart include:
HLHS most often is diagnosed through ultrasound before the baby’s birth. The cardiothoracic surgeons at Norton Children’s Heart Institute are poised to stabilize the newborn immediately while more diagnostic tests are performed.
In addition to administering prostaglandin to keep the PDA open, doctors will use medications and mechanical ventilation strategies (breathing assistance) to balance blood flow to the lungs and the rest of the body.
HLHS surgery may involve a series of three surgeries. In some cases, a child with hypoplastic left heart syndrome may need a heart transplant because the case is so complex or because the heart becomes weak after HLHS surgery.
Growing into adulthood with this unique single-ventricle circulatory system can come with complications and risks affecting the liver, kidneys, heart or lungs.
The Norton Children’s Heart Institute Fontan Clinic provides regular follow-up and monitoring to help spot any emerging issue early and treat it sooner, when more options may be available.
The clinic works in conjunction with the Norton Children’s Heart Institute’s adult congenital heart disease program to provide sophisticated care to patients born with heart conditions.