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Marco Severino first recognized dextrocardia in 1643.
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In these patients, the liver may be midline, the spleen absent or multiple, the atrial morphology unclear, and the bowel malrotated.
Often, normally unilateral structures are duplicated or absent.
More than a century later, Matthew Baillie described the complete mirror-image reversal of the thoracic and abdominal organs in situs inversus.
Situs inversus is present in 0.01% of the population.
Situs inversus with dextrocardia is also termed situs inversus totalis because the cardiac position, as well as the atrial chambers and abdominal viscera, is a mirror image of the normal anatomy.
When situs cannot be determined, the patient has situs ambiguous or heterotaxy.
The 2 primary subtypes of situs ambiguous include (1) right isomerism, or asplenia syndrome, and (2) left isomerism, or polysplenia syndrome.
In classic right isomerism, or asplenia, bilateral right-sidedness occurs.
The terms levocardia and dextrocardia indicate only the direction of the cardiac apex at birth; they do not imply the orientation of the cardiac chambers.
In levocardia, the base-to-apex axis points to the left, and in dextrocardia, the axis is reversed.
Isolated dextrocardia is also termed situs solitus with dextrocardia.