Your Child's Hypoplastic Left Heart Syndrome

Your Child's Hypoplastic Left Heart Syndrome

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Your Child's Hypoplastic Left Heart Syndrome

Illustration of a normal heart.

Front view cross section of heart showing hypoplastic left heart syndrome: coarctation (narrowing) of the aorta, patent ductus arteriosus (PDA), small or absent mitral valve, small left ventricle, small or absent aortic valve, patent foramen ovale (PFO), and small ascending aorta. Arrows show blood flowing from left atrium to right atrium through PFO, and mixed blood going from right ventricle to pulmonary artery and into aorta through PDA.

Your child has a hypoplastic left ventricle. This means that the left ventricle is either too small or absent. The most common heart problem that includes a hypoplastic left ventricle is called hypoplastic left heart syndrome (HLHS). This sheet explains HLHS. It can also be used to learn about similar heart problems. Your child’s health care provider will explain as needed.

The Normal Heart

  • The heart is divided into 4 chambers. The 2 upper chambers are called atria. The 2 lower chambers are called ventricles. The heart has 4 valves. The valves open and close. They keep blood flowing forward through the heart.

  • In a normal heart, oxygen-poor blood from the body fills the right atrium. This blood flows into the right ventricle. The right ventricle pumps this blood through the pulmonary artery to the lungs. There, the blood receives oxygen. Oxygen-rich blood returns from the lungs and fills the left atrium. This blood then flows into the left ventricle. The left ventricle pumps this blood through the aorta. From there, it travels out to the body.

  • The ductus arteriosus is a normal part of a baby’s heart before birth. It’s a blood vessel that connects the pulmonary artery and the aorta. It lets blood flow between the 2 vessels. It normally closes shortly after birth. If it remains open, it’s called a patent ductus arteriosus (PDA).

  • The foramen ovale is also a normal structure in a baby’s heart before birth. It’s an opening in the wall (atrial septum) between the atria. It normally closes a few weeks after birth. If it remains open, it’s called a patent foramen ovale (PFO).

What Is Hypoplastic Left Heart Syndrome?

  • With HLHS, the left side of the heart didn’t develop properly. The left heart structures are too small or absent. This includes the left ventricle. There may also be coarctation (narrowing) of the aorta.

  • Because the left ventricle is too small, it can’t do its job. This means that oxygen-rich blood can’t be pumped to the body in the normal way.

  • After birth, the PFO and PDA allow blood to flow through the heart and reach the body:

    • The PFO allows oxygen-rich blood from the left atrium to flow through the atrial septum and mix with oxygen-poor blood in the right atrium. This causes mixed blood (blood with some oxygen) to flow into the right ventricle and be pumped through the pulmonary artery.

    • The PDA allows some of the mixed blood in the pulmonary artery to flow into the aorta. This carries some oxygen to the body. Because the blood contains less oxygen than normal, your child’s skin, lips, and nails may look blue. This is called cyanosis.

  • If the ductus arteriosus closes after birth, the heart can’t use this path to send oxygenated blood to the body. This causes the child’s organs to fail. At the same time, the lungs become filled with too much blood and fluid. This leads to a problem called congestive heart failure (CHF).

What Causes Hypoplastic Left Heart Syndrome?

HLHS is a congenital heart defect. This means your child was born with it. The exact cause is unknown. Most cases seem to occur by chance. Having a family member with a left-sided heart problem can make HLHS more likely.

What Are the Symptoms of Hypoplastic Left Heart Syndrome?

A child with HLHS develops severe symptoms shortly after birth. Symptoms can include:

  • Trouble breathing or rapid breathing

  • Poor feeding

  • Tiredness

  • Irritability

  • Cyanosis

  • Circulatory collapse (child becomes gray, cold, and the heart may stop beating)

How Is Hypoplastic Left Heart Syndrome Diagnosed?

  • HLHS may be detected with fetal echocardiography (fetal ultrasound) before a child is born. This test uses sound waves to form a picture of the baby’s heart. This test can be done when the mother is at least 16 weeks pregnant.

  • If HLHS isn’t seen before birth, signs may be found during a physical exam shortly after birth.

  • If a heart problem is suspected, your child will be referred to a pediatric cardiologist. This is a doctor who diagnoses and treats heart problems in children. To confirm a diagnosis of HLHS, several tests may be done. These include:

    • Chest X-ray. X-rays are used to take a picture of the heart and lungs.

    • Electrocardiography (ECG or EKG). The electrical activity of the heart is recorded.

    • Echocardiography (echo). Sound waves are used to create a picture of the heart and look for structural defects and other problems.

How Is Hypoplastic Left Heart Syndrome Treated?

  • HLHS is treated with surgery. This can involve either a series of 3 heart surgeries or, less commonly, heart transplantation. Both treatment options have risks and benefits. Your child’s cardiologist and surgeon will discuss them with you.

  • Newborns are given IV medication to keep the ductus arteriosus open. This lets oxygenated blood reach the body.

  • Your child may need a procedure called a balloon septostomy, if the PFO is too small. This may be done to help until a complete repair can be done. During this procedure, a catheter (thin, flexible tube) with a balloon on the end is used. It is guided through a blood vessel into the heart. The balloon is inflated to widen the PFO. Sometimes, a stent may be placed to keep the hole open. This allows more blood to mix freely between the atria. More oxygenated blood can then reach the body.

What Are the Long-term Concerns?

  • HLHS continues to be one of the hardest heart problems to treat. After surgery, your child may have ongoing heart problems. Medications may be needed to manage symptoms and improve heart function. Your child will need more surgery. In the long term, heart transplantation may be needed. Regular follow-up visits with the cardiologist are needed for the rest of your child’s life.

  • In many cases, children with HLHS can be active. How active will vary with each child. Ask the cardiologist what activities your child can do safely.

  • Your child may need to take antibiotics before having any surgery or dental work. This is to prevent infection of the heart or valves. This is called infective endocarditis. The cardiologist will give you instructions for this.