Children's Hospital Colorado
Colorado Fetal Care Center

Heterotaxy (Isomerism)

We specialize in the big things, the small things and everything in between.

U.S. News & World Report honor roll badge

Get Care

Would you like to learn more about us?
checked box icon Colorado Fetal Care Center
Do you have questions about this condition?
Call

What is heterotaxy (isomerism)?

Heterotaxy, also called isomerism, is a rare condition that is usually diagnosed in utero. Heterotaxy results in a different arrangement of organs in the chest and abdomen. There are different forms of heterotaxy and approximately 80% involve heart conditions of varying types and severity. Heterotaxy happens with some of the most complex structural heart diseases. Isomerism often affects other organs, including:

  • Stomach: position changes and increased feeding difficulties
  • Intestines: abnormal arrangement, twisting of intestines or underdevelopment
  • Spleen: no spleen or a small spleen and increased risk for infections
  • Liver: abnormal communications between the liver and other blood vessels or liver in the wrong location
  • Lungs: a genetic breathing condition called primary ciliary dyskinesia or other respiratory illnesses
  • Heart Rhythm: too fast or slow heart rates

What is the life expectancy for heterotaxy?

The long-term outlook for children with heterotaxy depends largely on how severely it affects their organs — especially the heart. Children with more complex heart conditions may face greater medical challenges, but advances in surgery and medical care make it possible to repair many forms of congenital heart disease. With the right care, many children with heterotaxy can survive into adolescence and adulthood; however, all will need lifelong medical follow-up.

What causes isomerism?

Heterotaxy happens because of changes in a child’s genes. Sometimes these changes are passed down from one or both parents, and other times they happen randomly. In families with a history of heterotaxy, it may be possible to screen for heterotaxy before birth; however, most times, experts don’t detect heterotaxy until second trimester ultrasounds or even after birth.

Who gets heterotaxy?

Heterotaxy is rare and can affect anyone, regardless of race, ethnicity or gender. It usually develops early in pregnancy while the baby’s organs are forming. In some cases, it runs in families, but often it happens without any family history.

How rare is heterotaxy?

Heterotaxy affects about 1 in every 10,000 newborns. Some experts suspect isomerism could be more common than that because they also believe it is underdiagnosed.

Next steps

Cartoon image of a patient and doctor having a telehealth call.

Compassionate care, wherever you are

We’re here when you need us. Telehealth appointments are available across every specialty, so you can get the high-quality care we’ve always offered from the comfort, privacy and convenience of home.

See if telehealth is right for you

 

Get to know our pediatric experts.

Camila Londono-Obregon, MD

Camila Londono-Obregon, MD

Cardiology, Cardiology - Pediatric, Pediatrics

Debnath Chatterjee, MD

Debnath Chatterjee, MD

Anesthesiology, Anesthesiology

Patient ratings and reviews are not available Why?

Traci Nivens, MD

Traci Nivens, MD

Ob/Gyn Obstetrics & Gynecology

Patient ratings and reviews are not available Why?

Hannah Elfman, MS,  CGC

Hannah Elfman, MS, CGC

Genetics, Obstetrics & Gynecology

Patient ratings and reviews are not available Why?