Specifically, hypoplastic left heart syndrome is a single ventricle heart defect. Although these conditions are rare, they account for nearly a quarter of all pediatric heart surgeries we perform at Duke. Our single ventricle program is the first of its kind in North Carolina.
Hypoplastic Left Heart Syndrome (HLHS)
Hypoplastic left heart syndrome is one of the most common types of single ventricle heart defects. In a child with HLHS, the left side of the heart develops abnormally, which prevents the heart from being able to pump oxygenated blood to the body. Complex congenital heart defect like HLHS require a lifetime of highly specialized, coordinated care that usually starts before birth. We know that you want the very best care for your child -- so do we. Duke’s pediatric heart doctors, surgeons, and other specialists are renowned for their expertise and breakthrough techniques that add up to high-quality, compassionate care for your little one.
About HLHS Care at Duke
Home Monitoring
Children can be especially vulnerable between operations -- known as the interstage period. We stay in close contact with you to see how your child is doing. We also provide equipment and teach you how to monitor vital signs at home. We show you how to identify warning signs, and we provide a way for you to contact us directly.
Advanced Operations to Restore Normal Heart Circulation
Your child may be eligible for new surgical approaches that help the heart function more normally, known as biventricular repair or conversion. These could replace one or more of the traditional heart operations (single ventricle palliation) that your child would otherwise need in their lifetime. We are the only center in North Carolina offering these options.
Heart Transplant
Some children with HLHS may need a heart transplant.
Pediatric heart surgery is performed at Duke University Hospital. Pre- and post-surgical appointments may take place at Duke Children's Health Center or at other clinics in the region.
Tests for HLHS
Fetal Imaging
Heart defects like HLHS are usually discovered before birth during a routine second-trimester ultrasound and then confirmed with more advanced imaging like targeted fetal ultrasound, fetal echocardiography, and MRI. These show the structure and function of your child’s heart in more detail.

Imaging after Delivery
Doctors may order additional tests to learn more about your child’s condition after birth. Echocardiograms, electrocardiograms, and cardiac MRI scans don’t require sedation and cause little to no discomfort. Children are usually sedated for cardiac catheterization.
- Echocardiogram: A special ultrasound captures moving images of your child’s heart to determine its chamber dimensions, shape, valve structures, and overall function.
- Electrocardiogram: Small electrodes are placed on the skin to record the heart’s electrical activity.
- Cardiac MRI: Radio waves, magnets, and a computer create still and moving images of your child’s overall heart structure, heart muscle function, and blood vessels.
- Cardiac catheterization: A small, flexible tube called a catheter is inserted into a large blood vessel and guided to the heart to see its structures from the inside. Contrast dye may be injected, and X-rays taken to capture detailed images of your child’s heart and blood vessels.
Duke is the best pediatric cardiology and pediatric heart surgery program in North Carolina, and one of the top 10 hospitals in the country for children with heart disease, according to U.S. News & World Report.