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Sometimes children with heterotaxy syndrome have dextrocardia syndrome.

In some cases, there may be a functioning spleen, but it may be divided into several smaller spleens (polysplenia). When the spleen is missing or doesn’t work correctly, patients have a more difficult time recovering from surgeries or infections (patients with heterotaxy may require multiple surgeries). This can cause many problems, because the spleen helps the body fight infections.

In some cases of heterotaxy syndrome, the spleen may not work correctly or may be missing entirely.

There may also be irregularities with the skeleton, central nervous system and urinary tract.This also may require surgical intervention. Some children with heterotaxy syndrome can have a very serious condition of the liver called biliary atresia.Many children with malrotation need abdominal surgery to correct it. With this problem the bowel can twist on itself (volvulus). The intestines may have malrotation, which is when the loops of bowel are lined up incorrectly.In addition, organs such as the stomach, intestines, liver and lungs may be in abnormal places in the chest and abdomen. All usually involve heart defects of varying types and severity. There are different forms of heterotaxy syndrome.

The beginning of the word (hetero-) means “different” and the end (–taxy) means “arrangement.” Heterotaxy syndrome is a rare birth defect that involves the heart and other organs.