Conditions + Treatments

Heterotaxy Syndrome in Children

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Heterotaxy syndrome is a rare condition in which the heart and/or organs in the abdomen are in abnormal places and may be formed incorrectly. The condition can affect the heart and lungs, and the organs in the abdomen, such as the stomach, intestines, liver and lungs.

There are two main forms of heterotaxy syndrome:

  • Asplenia: Patients can have holes in the walls that divide the right and left sides of the heart, problems involving the valve and connections that carry blood from the heart to the lungs, and problems involving the valves that connect the upper and lower chambers of the heart.
  • Polysplenia: With polysplenia, there are multiple small spleens rather than a single spleen, although these spleens may not be functional.

Patients with heterotaxy syndrome may require multiple surgeries to either fix defects or to redirect blood flow when defects cannot be fixed. All procedures require life-long follow-up with a cardiologist.

How Boston Children's Hospital approaches heterotaxy syndrome

Our team at Boston Children’s Hospital treats some of the most complex pediatric heart conditions in the world, and our success rate is among the highest in the nation among large pediatric cardiac centers. Learn more about treatments for heterotaxy syndrome.

Heterotaxy syndrome: Reviewed by Puja Banka , MD, and John Mayer, MD
© Boston Children's Hospital; posted in 2012

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