Dextrocardia epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, M.B.B.S. [[2]]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Claudia Hochberg, M.D.; Assistant Editor-In-Chief: Kristin Feeney, B.S. [[5]]

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.