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Hypoplastic Left Heart Syndrome (HLHS)

What is Hypoplastic Left Heart Syndrome?

Hypoplastic Left Heart Syndrome (HLHS) is a rare congenital heart defect. It is a type of single ventricle heart defect. In HLHS, the left side of the heart is underdeveloped, which results in the inability of the left side of the heart to effectively pump oxygen-rich blood to the rest of the body.

Causes of HLHS

The exact cause of HLHS is unknown. It is thought that genetics and environmental factors may play a role in the development of HLHS. In some cases, the cause may be related to a decreased flow of blood in the placenta during pregnancy, or the fetus may experience distress during delivery.

Symptoms of HLHS

The symptoms of HLHS depend on how severe the defect is. Common signs of HLHS may include:

  • Poor appetite
  • Failure to gain weight
  • Fast breathing
  • Difficulty feeding or sleeping
  • Tiring quickly
  • Difficulty breathing
  • Bluish skin color (cyanosis)
  • Heart murmur
  • Leg swelling
  • Excessive sweating

Diagnosis of HLHS

The diagnosis of HLHS may be made with an ultrasound or an echocardiogram (ECG). An ECG is an imaging study that uses sound waves to create pictures of the heart and a heart defect can be identified. If these tests do not clearly show the defect, an MRI may be used to confirm the diagnosis. In some cases, genetic testing may be performed to confirm the diagnosis.

Treatment of HLHS

The main goal of treatment for HLHS is to improve the baby's quality of life, as the defect is not curable. Treatment typically involves the use of medications, lifestyle modifications, and, in some cases, surgery. Surgery may consist of either a single ventricle palliation, a Norwood procedure, or a Fontan procedure, depending on the severity of the defect.

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