What is hypoplastic left heart syndrome?

What is hypoplastic left heart syndrome?

What is hypoplastic left heart syndrome?

Sometimes during pregnancy, the left side of the baby's heart does not grow properly. This causes a rare anomaly called hypoplastic left heart syndrome.

Normally, the right side of the heart pumps blood from the heart to the lungs, where it receives oxygen. Returning to the heart, the left side pumps oxygen-rich blood to the rest of the body.

The heart of a child with hypoplastic left heart syndrome cannot do this. The lower left chamber may be smaller than normal or absent altogether. The valves on the left side may not be working properly, or the main artery from the heart may not be as large as it should be.

A child with hypoplastic left heart syndrome may also have a hole between the left and right upper chambers of the heart. This is called an atrial septal defect and can result in too much blood flow to the lungs.

Signs of hypoplastic left heart syndrome may not appear until a few days after birth. During the first two days, the baby's heart can adjust to the standard state. If the left side of the heart cannot pump blood well to the rest of the body, the right side will do more work. But adaptation lasts only a few days.

A newborn baby has a blood vessel that connects the two sides of its heart. This is called a persistent ductus arteriosus, and it only stays open for a few days after birth. After that, it naturally closes. It is then that most children with a defect begin to show symptoms of a lack of oxygen-rich blood and overwork of the heart.

Cause

Doctors don't know why hypoplastic left heart syndrome occurs, but it's a hereditary condition. Some experts believe that what a mother eats, drinks, or comes into contact with during pregnancy may also increase the risk of her baby developing hypoplastic left heart syndrome. This may include smoking or drinking alcohol, or not taking prenatal vitamins with folic acid.

Symptoms and diagnosis

Heart defects can be detected on ultrasound in the second trimester of pregnancy. Thus, some parents discover it even before the baby is born.

In other cases, hypoplastic left heart syndrome is detected a few days after the birth of the child. Signs include:

  • Drowsiness or lack of desire to move
  • Cold hands and feet
  • Fast breathing or difficulty breathing
  • Gray or blue skin
  • Poor eating

When your doctor listens to your child's heartbeat, they may hear a heart murmur that sounds like a whistle. This is due to unusual causes of hypoplastic left heart syndrome.

If your doctor thinks your child may have hypoplastic left heart syndrome, they will order an echocardiogram. In this test, sound waves are used to create images of her heart on a video screen. It can show the chambers of the heart and track blood flow.

Treatment

It is important that hypoplastic left heart syndrome be diagnosed and treated immediately. Otherwise, your baby's organs won't get enough blood. This may shock him.

Treatment includes:

  • Medication: Your child may be given a medicine called alprostadil to keep the ductus arteriosus open. He may also need medication to strengthen his heart muscle, lower his blood pressure, and help the body get rid of excess fluid.
  • Feeding and breathing assistance: Your baby will be weak and will likely need fluids through a drip or feeding tube. A breathing device called a ventilator can also be used to make sure your child is getting enough oxygen.
  • Atrial septostomy. If your child does not have an atrial septal defect, your doctor may recommend this surgery. It creates an opening between the upper chambers of the heart to allow more blood to pass through.

A child with hypoplastic left heart syndrome may also have a series of heart repair surgeries. The first, called the Norwood procedure, is performed shortly after birth. This is a complex operation that creates a new aorta for your child and allows the right ventricle of the heart to pump all the blood into the body (after the canal is closed).

Two more operations usually follow to restore the heart and maintain normal blood flow. Timing depends on your baby's condition, but the next surgery, known as Glenn's surgery, is usually done when your baby is a few months old. The third operation is called the Fontan operation and is performed at the age of 3 or 4 years.

In some cases, a doctor may suggest a heart transplant. This will give your child a healthy heart, but it may take time to find a donor. She will also have to take medication for the rest of her life so that her body does not reject them.

Your baby will need lifelong care from a cardiologist who specializes in birth defects. Your child is also likely to need other surgeries and is more likely to have other heart problems such as irregular heartbeats and blood clots.

An infant who has undergone heart repair surgery may grow up physically weaker than other children and have developmental problems. He may need additional support at home and at school.


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