*Information supplied by the St. Louis Children’s Hospital. Source

Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a condition from birth involving a group of defects of the heart and large blood vessels. This condition is developed during the first 8 weeks of pregnancy.

With this condition the left side of the heart is small and underdeveloped, the affected areas involved:

  • Mitral valve. This valve controls blood flow between the left atrium and left ventricle.
  • Left ventricle. This is the lower left chamber of the heart. It pumps blood to the body.
  • Aortic valve. This valve controls blood flow from the left ventricle into the aorta, and then to the body.
  • Aorta. This is the large artery leading from the left ventricle to the body.

When this condition occurs the body does not receive enough blood from the heart, if any at all. Due to this, if an infant does not receive surgery to fix the defect they will not live long after birth.

Hypoplastic left heart causes

There is no clear reason that a child develops this defect. Though, congenital heart defects tend to occur more often in certain families.

Hypoplastic left heart syndrome symptoms

Babies with HLHS usually get symptoms shortly after birth:.

  • Blue color of the skin, lips, and nails (cyanosis)
  • Pale skin
  • Sweaty, clammy, or cool skin
  • Trouble breathing
  • Fast heart rate
  • Cold feet
  • Poor pulses in the feet
  • Poor feeding

How is hypoplastic left heart syndrome diagnosed?

Usually, the condition is discovered during pregnancy through an ultrasound. However, if it goes undiscovered, the infant after birth appears to have pale blue skin, or is limp or listless. The infant will be referred to a pediatric cardiologist, where the following tests could be performed to discover the defect:

  • Chest X-ray
  • Electrocardiogram (ECG)
  • Echocardiogram (echo)