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Heterotaxy syndrome can’t be prevented, but a lot can be done to improve and often completely repair children's hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of heterotaxy syndrome and other congenital heart defects (also often called, “congenital heart disease”).
If your child’s been diagnosed with heterotaxy syndrome, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
As part of normal development in the womb, the body must determine which side is right and which side is left. This ensures that organs usually on the left (for example, the spleen) are on the left, and organs usually on the right (for example, the liver) are on the right. Occasionally, the developing body has a problem in determining which side is right and which side is left (this is called “heterotaxy syndrome”).
With heterotaxy syndrome, the developing body may believe both sides are “left sides,” or that neither side is a “left” side. This issue with “sidedness” may cause a range of abnormalities in the body, including problems in the abdomen and chest.
- In the abdomen, heterotaxy syndrome can cause children to not have proper rotation of the abdominal organs. For example a child with heterotaxy syndrome could have a liver that goes across both sides instead of one side or have no spleen or multiple spleens.
- In the chest, heterotaxy syndrome can cause problems with the structure of the heart and lungs, and their connections both to each other and the blood vessels coming in and going out. The blood vessels are often affected by heterotaxy syndrome because the vessels coming back from the body are not able to properly attach themselves to the heart if it is not specified which side is which.
How Does the Heart Normally Work?
When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »
Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:
Heterotaxy syndrome is usually diagnosed before birth by your obstetrician or maternal-fetal medicine specialist using a fetal ultrasound, or shortly after birth through an ultrasound and echocardiogram (“echo”) — a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart.
Heterotaxy syndrome itself isn’t treated, but some of the abnormal connections with the heart and lungs may need corrective surgery. And, although it doesn’t matter which side the liver is on, children may need surgery to fix certain other aspects of the abdominal “sidedness” in order to prevent complications later.
With heterotaxy syndrome, there’s a wide range of cardiac abnormalities and need for surgical repair — every child is different. Many children need surgery, but some don’t. Surgery may range in severity from one operation for some to children who need a series of operations called “single ventricle palliation.”
Usually, if a child with heterotaxy syndrome needs surgery, heart surgery is the top priority. So Nemours Cardiac Center surgeons will most often step in to perform any necessary heart operations first before correcting any problems in the abdomen. Abdominal surgery can be delayed because most abdominal surgeries are performed to prevent problems later in life, not to urgently treat a particular problem.
The long-term effects of heterotaxy syndrome depend on the specific type of congenital heart defect and abdominal problems. Some children don’t need heart surgery — or any surgery at all.
If your child has heterotaxy syndrome, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.
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Information for Patients
Outpatient Services and Inpatient Units: (302) 651-6660
After 5 p.m. and Weekends:
Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690