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.

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