Medical Definition of Heterotaxy

  • Medical Author:
    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

Heterotaxy: The abnormal placement of organs due to failure to establish the normal left-right patterning during embryonic development. The morbidity (disease) and mortality (death) from heterotaxy are caused by congenital defects, especially congenital heart disease.

Mutations in the zinc finger transcription factor ZIC3 cause an X-linked form of heterotaxy and have also been identified in patients with isolated congenital heart disease.

Reviewed on 9/7/2018

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