FACT SHEET
Hypoplastic left-heart syndrome (HLHS) 

General
What is hypoplastic left-heart syndrome?
Hypoplastic left-heart syndrome (HLHS) is a condition in which the left side of the heart is underdeveloped, causing complications in the heart’s ability to pump oxygenated blood out to the body.

How common is it?
HLHS is a congenital heart problem affecting one in 5,000 babies. Texas Children's Heart Center performs approximately 25 HLHS palliative surgical procedures annually.

How serious is the problem?
Left untreated, HLHS is fatal, usually within the first few days or weeks of life.

How is it detected?
HLHS can be detected in utero, but due to the size or position of the baby, the ultrasound may miss it. Because babies receive oxygenated blood from the placenta while in the womb, most complications occur after birth. Symptoms include a bluish skin color, sweatiness, difficulty eating and rapid breathing.

Treatment
What surgical options are available?
The current approach involves a three-stage operation designed to re-route the blood from the lungs, through the right side of the heart, and out to the body by circumventing blockages on the left side.

Stage I (Norwood)
Typically, the first operation is performed within the first few days of diagnosis. To improve blood flow to the body, the main lung (pulmonary) artery and body artery (small aorta) are linked to make a new, larger aorta. A passage or shunt is created using soft plastic (Gore-Tex) to provide blood flow to the lungs. The wall separating the heart’s top two chambers is also removed.

Stage II (Glenn Shunt)
Normally performed between four and nine months of age, the second operation reduces the workload of the heart and removes the Gore-Tex shunt. Blood from the head, neck and upper body is routed to the lung arteries. This allows blood to flow into the lungs for oxygen

Stage III (Fontan)
The third operation is performed as the child begins to develop the need for more blood flow to the lungs. This surgical procedure is designed to separate the red (oxygenated) and blue (unoxygenated) blood and will improve the child’s energy levels. A wall is created in the right collecting chamber and the chamber is attached on the base of the lung artery.

Prognosis
What is the prognosis for a patient surgically treated for HLHS?
Over the past decade, advancing surgical techniques, cardiac intensive care for children and specialized nursing care have provided relief for babies born with HLHS. Unfortunately, there is no sure answer for HLHS children who have undergone all three surgeries.

Every year, Texas Children's Heart Center has an increasing number of HLHS children living healthy lives. Texas Children's Heart Center is committed to the continued care of these children into adulthood.

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To refer a patient or for more information contact Texas Children's Heart Center.

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