Heterotaxy Syndrome (Isomerism)

Also known as: heterotaxy, situs ambiguous

What is ​Heterotaxy Syndrome (Isomerism)?

Heterotaxy syndrome is a rare birth defect that involves the heart and other organs where a child is born with organs on the opposite side of the body from where they are commonly located. Having a heart on the right side of the chest instead of the left is just one of many situations that can occur. Other bodily organs can be impacted as well.


What causes heterotaxy syndrome (isomerism)?

Researchers aren’t exactly sure what causes heterotaxy syndrome. There appears to be a genetic component to the disease, where it is passed down through families. Infections or exposures to certain chemicals may also play a role.


What are the symptoms of heterotaxy syndrome (isomerism)?

A variety of symptoms can occur depending on which organs are involved. Since the heart is usually involved, symptoms such as blue skin from poor circulation, rapid breathing, sweating, poor weight gain or irregular heartbeat are common, among others.


What are heterotaxy syndrome (isomerism) care options?

Surgery is often needed to correct the problems related to heterotaxy syndrome. Medication may also be needed to ensure proper bodily function. Additional procedures may be needed later in life due to potential complications of the disease.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 1/10/2017 3:28:37 PM

From the Newsdesk

Meet James Enos, MD - The Heart Program at Nicklaus Children's Hospital
10/04/2017 — James Enos, MD of Nicklaus Children's Hospital is a pediatric cardiologist with The Heart Program.
Dr. Anthony Rossi, discusses the importance of EKG screenings for young athletes
10/04/2017 — The Heart Program provides care for more children with congenital heart disorders than any other hospital in Florida and has been ranked among the nation’s best for pediatric cardiology and heart surgery by U.S.News & World Report.