Hypoplastic Left Heart Syndrome (HLHS)

Hypoplastic left heart syndrome is the most common type of single ventricle heart defect. Although these conditions are rare, they account for nearly a quarter of the pediatric heart operations we perform at Duke. Our single ventricle program is the first of its kind in North Carolina.

Once a diagnosis of HLHS is confirmed, your child’s care team immediately begins working with you to create a tailored HLHS treatment plan for birth and beyond. Duke’s team of experts includes pediatric heart surgeons, cardiologists, interventional cardiologists, critical and intensive care experts, anesthesiologists, and many other specialists.

Newborn Care

Newborns with HLHS are cared for in our specialized Cardiac Neonatal Intensive Care Unit. An intravenous infusion of a medication called prostaglandin helps keep open a blood vessel connection (the patent ductus arteriosus) that normally closes soon after birth. This allows blood from the right side of the heart to be circulated to the body.

HLHS Surgery

Children with HLHS typically need several operations within their first few years of life. This stepwise series of surgeries called “single ventricle palliation” rearranges the plumbing of the heart so that the right ventricle can effectively do the work of two ventricles.

1. Norwood Operation: This first operation usually takes place within the first two weeks after birth. Surgeons make new connections in the heart so it can pump blood simultaneously to both the lungs and body. Our pediatric heart surgeons pioneered a new technique for the Norwood operation that reduces risk, increases patient survival from less than 88% to more than 97%, and is better tolerated by newborns.

2. Glenn Operation: Usually done when a baby is four to six months old, this operation changes the source of blood flow to the lungs, reduces the workload on the right ventricle, and improves the efficiency of the heart.
    
3. Fontan Operation: Usually done when a child is between two and four years old, this operation further modifies the source of blood flow to the lungs. The Fontan operation routes all venous blood returning from the body directly to the lungs. This increases oxygen in the blood to near-normal levels. The Fontan Program at Duke Children’s Hospital and Health Center offers advanced care to those who have undergone the Fontan procedure to help your child stay healthy as they grow.

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 may be taken to capture detailed images of your child’s heart and blood vessels.