Hypoplastic Left Heart Syndrome (HLHS)

In hypoplastic left heart syndrome, the left side of the heart—including the aorta, aortic valve, left ventricle and mitral valve—is underdeveloped. Blood returning from the lungs must flow through an opening in the wall between the atria called an atrial septal defect. The right ventricle pumps the blood into the pulmonary artery, and blood reaches the aorta through a patent ductus arteriosus.

A baby born with HLHS often seems normal at birth, but will show symptoms within a few days of birth as the ductus closes. Babies with this syndrome become ashen, have rapid and difficult breathing and have difficulty feeding. Without treatment, this heart defect is usually fatal within the first days or months of life.

Treatment Options

Although this defect is not correctable, some babies can be treated with a series of operations, or with a heart transplant. Until an operation is performed, the ductus is kept open by intravenous (IV) medication. Because these operations are complex and different for each patient, you need to discuss all the medical and surgical options with your child's doctor. Your doctor will help you decide which option is best for your baby.

If you and your child's doctor choose surgery, the surgery will be performed in several stages. The first stage, called the Norwood procedure, allows the right ventricle to pump blood to both the lungs and the body. It must be performed soon after birth. The final stage has many names including bidirectional Glenn, Fontan operation and lateral tunnel. These operations create a connection between the veins returning blue (oxygen-poor) blood to the heart and the pulmonary artery. The overall goal of the operation is to allow the right ventricle to pump only oxygenated blood to the body and to prevent or reduce mixing of the red (oxygen-rich) and blue blood. Some infants require several intermediate operations to achieve the final goal.

Some doctors will recommend a heart transplant to treat this problem. Although it does provide the infant with a heart that has normal structure, the infant will require lifelong medications to prevent rejection. Many other problems related to transplants can develop, and you should discuss these with your doctor.

Children with hypoplastic left heart syndrome require lifelong follow-up care by a pediatric cardiologist for repeated checks of how their heart is working. Virtually all children with HLHS will require heart medicines.

People with hypoplastic left heart syndrome, before and after treatment, are at risk for getting an infection on the heart's valves (endocarditis). To help prevent this, they'll need to take antibiotics before certain dental and surgical procedures.

More Blogs

Understanding Heart Screenings for Your Young…

November 18th, 2013 | Soccer. Baseball. Basketball. Gymnastics. If your child plans to participate in an organized sport activity, they’ll need more than just the required gear and equipment; They’ll also need a yearly physical exam. While an annual physical is fairly comprehensive, your physician may recommend additional screening for heart-related… Continue Reading

Cholesterol and Your Child

November 13th, 2013 | As adults, we know we need to watch our cholesterol in order to maintain good health. But did you know that children can have high cholesterol, too? As a parent, you can take steps today to monitor your child’s cholesterol and reduce your child’s risk of developing cardiovascular disease later in life. How Do I Know if My Child… Continue Reading

Newborn Heart Screenings: Peace of Mind

November 11th, 2013 | If you’re expecting a baby, you are hoping to leave the hospital with a healthy baby in tow. One way health care providers are helping ensure your baby is healthy and strong enough to go home is through routine newborn heart screenings. As part of Indiana law, all newborns are required to have blood oxygen levels tested before leaving… Continue Reading