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Hypoplastic left heart syndrome

Hypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). In hypoplastic left heart syndrome, the left side of the heart is critically underdeveloped.

If your baby is born with hypoplastic left heart syndrome, the left side of the heart can't effectively pump blood to the body, so the right side of the heart must pump blood both 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.


Symptoms Causes Risk factors Complications Prevention

Babies born with hypoplastic left heart syndrome usually are seriously ill immediately after birth. Hypoplastic left heart syndrome symptoms include:

  • Grayish-blue skin color (cyanosis)
  • Rapid, difficult breathing
  • Poor feeding
  • Cold hands and feet
  • Being unusually drowsy or inactive

In a baby with hypoplastic left heart syndrome, if the natural connections between the heart's left and right sides (foramen ovale and ductus arteriosus) are allowed to close, he or she may go into shock and may die. Signs of shock include:

  • Cool, clammy skin that may be pale or gray
  • A weak and rapid pulse
  • Abnormal breathing that may be either slow and shallow or very rapid
  • Dilated pupils
  • Lackluster eyes that seem to stare

A baby who is in shock may be conscious or unconscious. If you suspect your baby is in shock, immediately emergency number.

When to see a doctor

It's more 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:

  • Grayish-blue skin color
  • Rapid, difficult breathing
  • Poor feeding
  • Cold hands and feet
  • Being unusually drowsy or inactive

If your baby has any of the following signs of shock, call emergency number right away:

  • Cool, clammy skin that may be pale or gray
  • A weak and rapid pulse
  • Abnormal breathing that may be either slow and shallow or very rapid
  • Dilated pupils in the eyes
  • Lackluster eyes that seem to stare

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