Children's Hospital Colorado

Heterotaxy Syndrome

What is heterotaxy syndrome?

Heterotaxy is a rare birth defect that results in an abnormal arrangement of the heart and other organs in the body. There are different forms of heterotaxy and approximately 80% involve heart defects of varying types and severity. Other organs are often affected in heterotaxy, including:

  • Stomach
  • Intestines
  • Spleen
  • Liver
  • Lungs

How is heterotaxy syndrome diagnosed?

Prenatal ultrasound can identify babies with heterotaxy syndrome before birth due to abnormal organ arrangement or structural heart problems in the baby.

Treatment for heterotaxy syndrome

Many babies with heterotaxy syndrome will require surgery shortly after birth and delivery at a high-risk center is recommended.


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