Dextrocardia epidemiology and demographics


 * Associate Editors-In-Chief: Priyamvada Singh, M.B.B.S. mailto:psingh@perfuse.org; ; Keri Shafer, M.D. [mailto:kshafer@bidmc.harvard.edu]; Claudia Hochberg, M.D.; Assistant Editor-In-Chief: Kristin Feeney, B.S. mailto:kfeeney@perfuse.org

Overview
Dextrocardia is categorized as a fairly rare disease. The reported likelihood of occurrence is roughly 1 in 100 births with approximately 1 in 1,000 of these cases to have dextrocardia and situs inversus.

Epidemiology
Dextrocardia is believed to occur in approximately 1 in a 100 people, while 1 in 1,000 of these will have Situs Inversus. The most recent statistics place the incidence of stitus inversus to be 1:2500 to 1:20,000. It is significantly less common to have dextrocardia with situs solitus.

Kartagener’s syndrome occurs in approximately 1 in 15-25 of patients with Totalis (situs inversus with dextrocardia). Kartagener Syndrome (KS), is a rare autosomal recessive genetic disorder caused by a defect in the action of the tiny hairs (cilia) lining the respiratory tract. Specifically, it is a defect in a gene coding for left-right dynein (lrd), a key structural protein in cilia.