Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
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Home / A–Z Services / Pediatric Heart Institute / Heart Conditions / Hypoplastic Left Heart Syndrome (HLHS)
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Hypoplastic Left Heart Syndrome (HLHS)


What is Hypoplastic Left Heart Syndrome?

Hypoplastic left heart syndrome is a series of heart defects that affect the left side of a newborn's heart. The cause is unknown. However, we do know that it develops during gestation. The lower left chamber of the heart (left ventricle) is small and therefore cannot adequately pump blood through the aorta out to the body. Additionally, the aortic and mitral valves do notwork properly and the aorta is much smaller than normal.

How do we diagnose Hypoplastic Left Heart Syndrome?

For the first several days of life, a newborn will have a patent ductus arteriosus (PDA). This allows blood to be pumped from the right ventricle to both the lungs and the body. However, when this closes (within the first five days life), the right side has no way of pumping the blood to the body and the left ventricle and aorta are too small to provide an adequate blood supply. Following this, the newborn will begin to exhibit symptoms such as shortness of breath, rapid breathing, pounding heart, bluish skin from lack of circulation, cold arms and legs, poor pulses, difficulty feeding, and overall lethargy.

In recent years, there has been an increased ability to detect this defect during the gestational period by ultrasonography. During the newborn period, an echocardiogram can usually identify this defect.

How do we treat Hypoplastic Left Heart Syndrome?

Treatment usually consists of a series of heart surgeries or a heart transplant. If surgical intervention is recommended, there are typically three procedures involved.

Norwood operation: This surgery is performed within the first two weeks of life. The surgeon will reconstruct the aorta and connect it directly to the heart's right ventricle. This will allow better blood flow out to the body. Your child may still appear slightly blue because the oxygen-rich and oxygen-poor blood mix in the right ventricle.

Bidrectional Glenn shunt or hemi-Fontan procedure: This is the second stage, usually happening around four to six months of life. This procedure involves connecting the superior vena cava directly to the lungs. It will reduce the work load on the right ventricle and allow it to pump blood mainly through the aorta. Additionally, because most of the oxygen-poor blood is being routed directly to the lungs, more oxygen-rich blood is being pumped out to the body. However, your infant will still appear blue because there is some oxygen-poor blood entering the right side of the heart.

The Fontan: This procedure is done sometime between 18 months of age to three years of age. During this surgery, the inferior vena cava is connected directly to the lungs. This prevents oxygen-poor and oxygen-rich blood from mixing and uses the right ventricle to pump oxygen-rich blood out to the body through the aorta. Following this procedure, the right ventricle is the only pumping chamber of the heart and your child will no longer appear blue.

Last Edited: June 30, 2016
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