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Heart Chambers and Valves  
heart anatomy

Copyright © Nucleus Medical Media, Inc.

Blood Flow Through the Heart  

Copyright © Nucleus Medical Media, Inc.

Definition  

Hypoplastic left heart syndrome is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood then moves out to the rest of the body.

With this syndrome, structures on the left side of the heart, which includes the aorta, aortic valve, left ventricle, and mitral valve, may be:

  • Too small
  • Absent
  • Abnormally developed

Since the heart cannot function properly, oxygen-rich blood flow to the body is limited. This condition requires immediate care from a doctor.

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Causes  

This is a congenital heart defect. This means that the heart forms incorrectly when the baby develops in the womb. The baby is born with the condition. It is not known why the heart forms this way.

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Risk Factors  

These factors increase your chance of having a child with hypoplastic left heart syndrome:

  • Previous pregnancy with fetal heart abnormalities or fetal loss
  • Family history of congenital heart defect
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Symptoms  

Symptoms usually appear within days after birth. Tell the doctor if you notice the following in your infant or child:

  • Blue/gray skin color
  • Cool skin
  • Rapid or difficult breathing
  • High heart rate
  • Sweaty, clammy skin
  • Poor feeding
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Diagnosis  

You will be asked about your child's symptoms and medical history. A physical exam will be done.

Images may be taken of your child's chest. This can be done with:

Other tests may be done to evaluate your child's heart. This can be done with:

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Treatment  

Talk with your doctor about the best treatment plan for your child. Some defects may be so severe that they are difficult to treat. Treatment options include:

Medications  

Medications are necessary to keep blood flowing through the ductus arteriosus. The ductus arteriosus is a connection between the pulmonary artery and the aorta, which is the largest artery in the body. It usually closes within a few days after birth. Keeping this passage open is a temporary treatment. Other medications may be used as well.

Surgery  

Surgery may be done to improve blood flow. This can be done through a variety of reconstructive and shunting procedures. Surgeries are usually done in stages:

  • After birth
  • 4-6 months of age
  • 2-4 years of age

Lifelong Monitoring  

Your child will need to see a heart specialist regularly. Heart medication will be needed throughout your child's life.

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Prevention  

There is no way to prevent this condition. Getting proper prenatal care is always important.