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Genetic and Rare Diseases Information Center (GARD)

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Situs inversus


Other Names for this Disease
  • Complete situs inversus
  • Complete situs inversus viscerum
  • Situs inversus totalis
  • Situs inversus viscerum
  • SIV
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

Can you provide me with information about situs inversus?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is situs inversus?

Situs inversus is a condition in which the arrangement of the internal organs is a mirror image of normal anatomy. It can occur alone (isolated, with no other abnormalities or conditions) or it can occur as part of a syndrome with various other defects. Congenital heart defects are present in about 5-10% of affected people. The underlying cause and genetics of situs inversus are complex. Familial cases have been reported.[1][2]
Last updated: 6/1/2015

What are the signs and symptoms of situs inversus?

In isolated situs inversus, there is a complete mirror image transposition of the thoracic (chest) and abdominal organs, and anterior-posterior (front-back) symmetry is normal. Many affected people have no associated health issues when the condition is isolated. When situs inversus occurs in association with other conditions such as Kartagener syndrome or primary ciliary dyskinesia, additional signs and symptoms relating to these conditions will be present.[1]
Last updated: 6/1/2015

What other symptoms or conditions may be associated with situs inversus?

Dextrocardia can occur with situs inversus. Dextrocardia means that the heart lies on the right side of the body, and the apex (tip) of the heart points to the right instead of the left. Situs inversus can also occur in association with syndromes such as Kartagener syndrome or primary ciliary dyskinesia (PCD). There may be incomplete transposition of thoracic or abdominal organs, as well as additional anomalies such as congenital heart disease, polysplenia (more than one spleen), asplenia (absent spleen), annular (ring-shaped) pancreas, horseshoe kidney, and diaphragmatic hernia.[1]
Last updated: 11/13/2014

How is situs inversus diagnosed?

A thorough physical examination, followed by radiographic imaging of the chest and abdomen and electrocardiography, identify most cases of situs inversus. The main diagnostic challenge in affected people is the non-traditional presence of referred pain (pain felt in a different location than its source).[1]
Last updated: 6/1/2015

How common is situs inversus?

The incidence of situs inversus is approximately 1 in 10,000, with slightly more affected males than females.[1]
Last updated: 6/1/2015

Is situs inversus inherited?

The genetics of situs inversus is complex. Several familial cases have been reported in which the inheritance has been described as either autosomal recessive (most commonly), autosomal dominant, or X-linked. The condition appears to be genetically heterogeneous, meaning that different genetic factors or genes may cause the condition among different people or families.[1] If situs inversus is associated with another underlying syndrome or condition, the inheritance pattern may be the same as that of the underlying condition.

People with questions about genetic risks to themselves or family members are encouraged to speak with a genetics professional.
Last updated: 6/1/2015

How might situs inversus be treated?

In isolated situs inversus, no treatment may be necessary. When situs inversus is associated with another condition, treatment may depend on the associated condition and the signs and symptoms present in the affected person.[1]

Knowing that a person has situs inversus is important for diagnosing medical problems and preventing surgical mishaps that can result from the failure to recognize reversed anatomy. For example, in a person with situs inversus, appendicitis causes pain in the left lower abdomen instead of the right lower abdomen.[3] Wearing medical identification can help ensure proper treatment in an emergency medical situation.
Last updated: 6/1/2015

References
  • Tiller GE, Hamid R. Situs Inversus. NORD Guide to Rare Disorders. 2003;
  • Talabi AO, Sowande OA, Tanimola AG, Adejuyigbe O. Situs inversus in association with duodenal atresia. Afr J Paediatr Surg. July-September, 2013; 10(3):275-278.
  • Annamaria Wilhelm. Situs Inversus Imaging. Medscape. October 7, 2013; http://emedicine.medscape.com/article/413679-overview#showall.
Other Names for this Disease
  • Complete situs inversus
  • Complete situs inversus viscerum
  • Situs inversus totalis
  • Situs inversus viscerum
  • SIV
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.