What is a visceral heterotaxy?
DEFINITIONS. Heterotaxy — Heterotaxy, derived from Greek (hetero, meaning “different,” and taxy, meaning “arrangement”), is also referred to as visceral heterotaxy or heterotaxy syndrome. It is defined as an abnormal arrangement of the internal thoracic-abdominal organs across the left-right axis of the body.
How common is heterotaxy syndrome?
Heterotaxy syndrome affects a reported 1 in 10,000 births worldwide. However, some researchers believe it is more common than that because the condition is sometimes diagnosed later in life.
Is Heterotaxy genetic?
Inheritance. Most often, heterotaxy syndrome is sporadic, meaning that only one person in a family is affected. However, about 10 percent of people with heterotaxy syndrome have a close relative (such as a parent or sibling) who has a congenital heart defect without other apparent features of heterotaxy syndrome.
How long can you live with heterotaxy?
Survival for patients with heterotaxy syndrome was 83% over a median follow-up of 65 months. Thirty-four per cent of patients had a poor outcome.
How is Heterotaxy diagnosed?
It is often diagnosed before birth, when an ultrasound identifies an abnormal position of one or more organs. Sometimes, heterotaxy is diagnosed with a fetal echocardiogram (a special ultrasound of the heart before birth) when a complex heart defect is identified.
What is heterotaxy?
Heterotaxy syndrome is a rare birth defect that involves the heart and other organs. The beginning of the word (hetero-) means “different” and the end (–taxy) means “arrangement.” There are different forms of heterotaxy syndrome. All usually involve heart defects of varying types and severity.
What is heterotaxy syndrome?
Heterotaxy syndrome is an arrangement of internal organs somewhere between situs solitus and situs inversus; this condition is also known as “situs ambiguus.” Unlike situs inversus, the abnormal arrangement of organs in heterotaxy syndrome often causes serious health problems.
What is the difference between situs inversus and heterotaxy?
Unlike situs inversus, the abnormal arrangement of organs in heterotaxy syndrome often causes serious health problems. Heterotaxy syndrome can alter the structure of the heart, including the attachment of the large blood vessels that carry blood to and from the rest of the body.
How is heterotaxy diagnosed?
Heterotaxy is typically diagnosed by imaging of the internal organs through a CT scan or an MRI. More specific imaging of the heart such as an echocardiogram or an electrocardiogram may also be used to diagnose the condition. If heterotaxy is suspected, further tests may be completed to check for the functioning of the internal organs.
What is the prognosis of heterotaxy?
Prognosis Prognosis. The long-term outlook for people affected by heterotaxy depends on the specific organs that are affected in each individual. When children are diagnosed with heterotaxy soon after birth, it is typically because there are heart defects that require immediate surgery.