Heterotaxy Syndrome

This is not a CHD but people with Heterotaxy Syndrome also have a congenital heart defect.

What is it?

Heterotaxy Syndrome is a complex syndrome that occurs when the axes of the body fail to rotate correctly when developing in the womb. This can result in many different organ systems being affected, and also means that each individual with Heterotaxy is unique. Many individuals have a variety of severe congenital heart disease, while a smaller percentage have minor heart problems or none at all. Some have lung complications, while for others the major concern is their gastro-intestinal or immune system.

Heterotaxy syndrome Credit- congenital-heart-defects.co.uk:

Heterotaxy Syndrome is known by several different terms. It can be referred to as situsinversus ambiguous. (Note: situsinversustotalis is a complete reversal of organ placement with no functional abnormalities.) It is also known as Ivemark’s Syndrome, after the doctor who first recognized the pattern of abnormalities. Heterotaxy Syndrome is sometimes called right or left atrial isomerism as well. It often appears that the individual has either two right sides or two left sides, as if the body attempted to make itself symmetrical. Since our internal organs are not symmetrical, this causes several anomalies. It is often classified based on the number or lack of spleens.

Contact

Please note: Children’s Heart Surgery Fund is not a medical resource and cannot provide medical advice. Heart conditions are very individual and what can be right for one person could potentially be dangerous for another. If you have any questions about yours or your child’s condition, please get in touch with Leeds Congenital Heart Unit directly, or the specialist nurses.

 If you have any questions about your or your child’s heart condition, please contact the Cardiac Nurse Specialists

Children’s Cardiac Nurse Specialists

Phone number: 0113 3925467

Email: ccns.lgi@nhs.net

Adult Cardiac Nurse Specialists

Phone number: 0113 3928154
Email: leedsth-tr.achdnurse@nhs.net