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: 6/12/2018 1:29:13 PM

From the Newsdesk

Renowned Congenital Heart Surgeon, Dr. Kristine J. Guleserian, Honored with the 2018 AGBU New England District’s Global Excellence Award
06/19/2018 — Renowned congenital heart surgeon, Dr. Kristine J. Guleserian of the Nicklaus Children’s Heart Program was honored with the 2018 AGBU New England District’s Global Excellence Award for her achievements and work in congenital heart surgery and heart transplantation in Dallas, Miami and throughout the world.
June Patient of the Month: Victoria
06/01/2018 — Meet Victoria, our June Patient of the Month! Before birth, Victoria, was diagnosed with hypoplastic left heart syndrome (HLHS). While Victoria's mother was pregnant with her, the left side of her heart did not form correctly, causing an urgent need for surgery just after birth to help restore its function, or Victoria would die. Fast-forward to today, and Victoria, now a teenager, is leading a healthy life with her family in South Florida. She enjoys music and playing basketball with her siblings.

Video

video
Dr. David Drossner, a pediatric cardiologist with The Heart Program at Nicklaus Children's Hospital, discusses how congenital heart disease (CHD) is identified prenatally.