Hypoplastic left heart syndrome
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Hypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). The left side of the heart is critically underdeveloped in hypoplastic left heart syndrome.
If your baby is born with hypoplastic left heart syndrome, the left side of the heart can’t effectively pump blood to the body. Instead, the right side of the heart must pump blood to the lungs and to the rest of the body.
Medication to prevent closure of the connection (ductus arteriosus) between the right and left sides, followed by either surgery or a heart transplant, is necessary to treat hypoplastic left heart syndrome. With advances in care, the outlook for babies born with hypoplastic left heart syndrome is better now than in the past.
Babies born with hypoplastic left heart syndrome usually are seriously ill soon after birth. Hypoplastic left heart syndrome symptoms include:
If the natural connections between the heart’s left and right sides (foramen ovale and ductus arteriosus) are allowed to close in the first few days of life in a baby with hypoplastic left heart syndrome, he or she can go into shock and may die. Signs of shock include:
A baby who is in shock may be conscious or unconscious. If you suspect your baby is in shock, immediately call 911 or your local emergency number.
It’s likely that your baby would be diagnosed with hypoplastic left heart syndrome either before birth or soon after. However, you should seek medical help if you notice that your baby has the following symptoms:
If your baby has any of the following signs of shock, call 911 or your local emergency number right away:
A normal heart has two upper and two lower chambers. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood out of your heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings.
Hypoplastic left heart syndrome occurs during fetal growth when the baby’s heart is developing. The cause is unknown. However, if your family has one child with hypoplastic left heart syndrome, the risk of having another with a similar condition is increased.
A normal heart has four chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout the body — the heart uses its left and right sides for different tasks.
The right side moves blood to the lungs. In the lungs, oxygen enriches the blood, which then circulates to the heart’s left side. The left side of the heart pumps blood into a large vessel called the aorta, which circulates the oxygen-rich blood to the rest of the body.
In this condition, the left side of the heart — including the aorta, aortic valve, left ventricle and mitral valve — is underdeveloped (hypoplastic). As a result, the body doesn’t receive enough oxygenated blood.
In hypoplastic left heart syndrome, the left side of the heart can’t properly supply blood to the body because the lower left chamber (left ventricle) is too small or in some cases may not even exist. In addition, the valves on the left side of the heart (aortic valve and mitral valve) don’t work properly, and the main artery leaving the heart (aorta) is smaller than normal.
For the first several days of life, the right side of the heart can pump blood both to the lungs and to the rest of the body through a blood vessel that connects the pulmonary artery directly to the aorta (ductus arteriosus). The oxygen-rich blood returns to the right side of the heart through a natural opening (foramen ovale) between the right chambers of the heart (atria). When the foramen ovale and the ductus arteriosus are open, they are referred to as being “patent.”
If the ductus arteriosus and the foramen ovale close — which they normally do after the first day or two of life — the right side of the heart has no way to pump blood out to the body. Keeping these connections open with medication is necessary for survival in the first few days of life in babies with hypoplastic left heart syndrome. This will keep blood flowing to the body until heart surgery is performed.
If you already have a child with hypoplastic left heart syndrome, you have a higher risk of having another baby with this condition or a similar condition.
Beyond family history, there are no clear risk factors for hypoplastic left heart syndrome.
Without surgery, hypoplastic left heart syndrome is fatal, usually within the first few weeks of life.
With treatment, many babies survive, although most will have complications later in life. Some of the complications may include:
There’s no known cause of hypoplastic left heart syndrome, so there’s no known way to prevent the condition. If you have a family history of heart defects, or if you already have a child with a congenital heart defect, before getting pregnant consider talking with a genetic counselor and a cardiologist experienced in congenital heart defects.
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Hypoplastic left heart syndrome
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