Hypoplastic left heart syndrome (HLHS) is a congenital (present at birth) heart defect in which the left side of the heart is small and underdeveloped (hypoplastic). The left ventricle, mitral valve, aortic valve and aorta are all underdeveloped, and the left side of the heart is unable to send enough blood with oxygen to the body. This results in the left side of the heart being unable to support the circulation needed by the body's organs.

This condition is more common in males than in females and has no known cause.

HLHS is one of the most challenging and complex congenital heart diseases treat and manage. Without treatment, this defect is usually fatal within the first weeks of life. The University of Maryland Children's Heart Program is highly experienced in diagnosing and surgically treating the hearts of babies born with HLHS so that they can live longer lives.

Diagnosing HLHS

HLHS is often diagnosed by fetal ultrasound before the baby is born. Sometimes HLHS is not detected before birth. Those babies may initially appear healthy, although their oxygen levels will be slightly decreased. They may develop difficulty breathing, have fast breathing, have poor feeding or have cool and pale feet and hands. If HLHS or another heart defect is suspected, the baby will be evaluated by a pediatric cardiologist.

That evaluation would include:

  • measuring the oxygen level
  • having an electrocardiogram (ECG)
  • having an echocardiogram (ultrasound of the heart)

The echocardiogram would show the abnormal structure of the heart.

Providers

Treating HLHS requires a team of highly trained providers. The University of Maryland Children's Heart Program team includes a pediatric cardiac surgeon, pediatric cardiologists, pediatric and neonatal intensive care doctors and nurses, pediatric cardiac anesthesiologists, cardiac operating room staff, cardiac nurses and many others with expertise in providing this complex and highly specialized care.

Treating HLHS

The immediate treatment for newborns with HLHS is to ensure that the blood returning from the lungs has a way to get to the body. Babies are started on an IV medication to keep the connection between the pulmonary artery and the aorta open. This allows the right ventricle to be the pump to both the lungs and the body.

This treatment helps stabilize the baby for surgery, but does not provide a long-term solution.

Patients with HLHS must undergo the following three palliative surgeries within the first few years of their lives.

Norwood

The first surgery, the Norwood operation, happens in the first week or so of your baby's life.

This procedure is an open heart surgery that accomplishes three goals:

  • Getting blood to the lungs
  • Getting blood to the body
  • Making sure that the blood returning from the lungs gets to the right side of the heart

The surgeon attaches the pulmonary artery and the aorta to create one large blood vessel that goes from the right ventricle to the body. This means that the branches of the pulmonary artery that go out into the lungs are no longer attached to the heart. A tube is placed that connects either the right ventricle or the aorta to the pulmonary artery branches and creates a stable way to get blood to the lungs. Finally, the wall between the right and left atria is cut out to allow all the blood coming back from the lungs to get to the right side of the heart.

The Norwood procedure accomplishes the three goals, but it is not a final solution. The right ventricle still has to do extra work by pumping blood to both the lungs and the body, and the body gets blood that is a mixture of oxygen rich and oxygen poor blood instead of all oxygen rich blood. Also, the tube that gets blood to the lungs will not grow as your baby grows and will eventually need to be removed.

Within a few months of the Norwood operation, your child will need to have a Glenn shunt procedure.

Glenn Shunt

When your baby is 4 to 6 months of age, he or she will have the Glenn shunt procedure. This operation is the first step of separating the lung and body circulations. 

This surgery involves detaching the large vein that drains the oxygen-poor blood from the head and upper body into the right atrium (called the superior vena cava, or SVC) from the heart and attaching it directly to the pulmonary arteries. This procedure will let the blood drain directly into the lungs. The tube from the Norwood operation that connects the right ventricle or aorta to the pulmonary artery is then removed.

After this procedure, the right ventricle only has to pump the blood to the body, so it doesn't have to work as hard. However, all of the oxygen poor blood from the lower body is still coming into the right atrium by another large vein (called the inferior vena cava, or IVC) and mixing with the blood from the lungs. This means that the blood going to the body still has a decreased oxygen level.

Withing a few years of this procedure, your child will need a Fontan operation.

Fontan

The final procedure, called the Fontan operation, takes care of the blood that has a decreased oxygen level. This surgery is usually performed around 2 to 4 years of age. In this surgery, the IVC is removed from the heart and connected to the pulmonary arteries. At this point, all of the oxygen poor blood will drain directly into the lungs, and the right ventricle will pump oxygen rich blood to the body.

Recovering from this procedure can take two or three weeks in the hospital, where your child will receive continual care, and you will learn how to manage HLHS.

View our pediatric cardiac surgery outcomes.

Long-term Outcome for Children with HLHS

Children with HLHS will need lifelong follow-up with a cardiologist. When your child is young, follow-up visits are frequent. After they have completed the series of surgeries, they will be seen every 6 to 12 months. At follow-up visits, children will get regular ECGs and echocardiograms. Other tests, such as cardiac MRIs, exercise stress tests or Holter (24 hour ECG) monitors may be also be performed.

Survival for infants with HLHS has increased as we continue to improve our techniques and approaches to the management of this complicated disease. Surgery for HLHS was first performed only a few decades ago. There are now adolescents and young adults with HLHS who had successful surgeries when they were infants.

Patients with HLHS may need to take daily medications and may have some limitations in their exercise ability. Despite this, they are often able to lead happy and productive lives. As we develop new technologies and ways of approaching this disease, we expect that infants born today with HLHS will have even better outcomes.

To make an appointment with a UM Children's Heart Program physician, please call 410-328-4348.

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