Hypoplastic Left Heart Syndrome

(HLHS)

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.
Heart Chambers and Valves
heart anatomy
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Blood Flow Through the Heart
IMAGE
Copyright © Nucleus Medical Media, Inc.

Causes

Hypoplastic left heart syndrome is present at birth. It is not known exactly why the heart does not develop normally.

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 defects

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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