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Genetic and Rare Diseases Information Center (GARD)

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Isolated levocardia


Other Names for this Disease

  • Isolated levocardia with situs inversus
  • Situs inversus with levocardia
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

I am pregnant and my baby has been diagnosed with isolated levocardia with situs inversus. She does not appear to have a heart defect. I've found little information and no support groups for this specific condition. I was given a very grim statistic for her chance of long-term survival. Is this true? Has there been other cases of prenatally diagnosed isolated levocardia with situs inversus? Was MRI or other tests helpful in planning the infants care?

Our Answer

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

What is isolated levocardia?

Isolated levocardia is a type of situs inversus where the heart is located in the normal position, but there is a mirror-image reversal of other internal organs.[1] Isolated levocardia may occur alone or with heart defects, heart rhythm abnormalities (sick sinus syndrome or atrioventricular node disorder), spleen defects (absent, underdeveloped, or extra spleen), and intestinal malrotation. Long term outlook varies depending on the presence/absence of associated abnormalities, particularly heart defects. The cause of isolated levocardia is not known. It is not usually associated with chromosome abnormalities.[7363]

Last updated: 3/10/2014

Are there any support resources for isolated levocardia?

While we could not find a support resource specifically for families with a child with isolated levocardia, there are a number of organizations that provide information, networking opportunities, and support to families with a child with a heart condition. You can view these groups on our Web site at the following link:
 http://rarediseases.info.nih.gov/gard/12032/isolated-levocardia/resources/5
Last updated: 3/10/2014

What is the long-term outlook of infants prenatally diagnosed with isolated levocardia?

The overall outlook for infants born with isolated levocardia will vary depending largely on the absence/presence of heart defects. You can find grim estimates for long term survival in dated literature (published in the 1960’s and 70’s). These statistics do not account for mild cases of isolated levocardia diagnosed as a result of improved technologies and detection.[2][1]

Last updated: 3/10/2014

Have there been other cases of prenatally diagnosed isolated levocardia?

The actual prevalence of prenatally diagnosed cases of isolated levocardia is not known. Our search of the medical literature identified nine published cases.
Last updated: 3/10/2014

Has MRI or other tests been helpful in planning the care of infants prenatally diagnosed with isolated levocardia?

Yes. In isolated levocardia it can be difficult to determine the position of the internal organs. Ultrasonography, CT, and MRI have been used alone and in combination to improve imaging of the internal organs and major blood vessels.[2][1]

In addition, a careful assessment of the spleen in the newborn is important. People with spleen dysfunction are at an increased risk for serious infection and benefit from prophylactic life-long antibiotics and vaccination. Barium contrast screening has been used for early detection of intestinal malrotation and to guide treatment. Also, long-term, infrequent follow-up of infants and adults with isoalted levocardia to monitor for heart rhythm problems is recommended.[2][1]
Last updated: 3/10/2014

References
  • Ghawi H1, Zghouzi MM, Emahbes TM, Awad SM. Prenatal diagnosis of isolated levocardia and a structurally normal heart: two case reports and a review of the literature. Pediatr Cardiol. 2013 Apr; 34(4):1034-7. http://www.ncbi.nlm.nih.gov/pubmed/?term=Ghawi%5Bau%5D+levocardia. Accessed 3/10/2014.
  • Gindes L, Hegesh J, Barkai G, Jacobson JM, Achiron R. Isolated levocardia: prenatal diagnosis, clinical importance, and literature review. J Ultrasound Med. 2007 Mar; 26(3):361-5. http://www.ncbi.nlm.nih.gov/pubmed/17324986. Accessed 3/10/2014.
Other Names for this Disease
  • Isolated levocardia with situs inversus
  • Situs inversus with levocardia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.