Key result
Heterotaxy syndrome with right atrial isomerism is associated with primary asplenia in over 50% of cases and has a worse prognosis than left atrial isomerism.
Why the study?
Heterotaxy syndrome presents with variable spleen anatomy affecting infection risk and management, but distinctions between forms are not well characterized.
Heterotaxy syndrome is a rare multiorgan disorder with right atrial isomerism being the most frequent form and associated with worse prognosis and primary asplenia.
Warrants asplenia screening in right isomerism heterotaxy; leaves open optimal management pending higher-level evidence.
Heterotaxy syndrome (HS) is a rare multiorgan disorder characterized by congenital heart defects (CHDs) and abnormal arrangement of the internal organs of chest and abdomen. Asplenia, polysplenia, hyposplenia, or ectopic spleens are frequently observed.1 HS is divided into 2 forms: right atrial isomerism, which is associated with worse prognosis compared with the second form: left atrial isomerism. HS with right atrial isomerism is associated in more than 50% of the cases with primary asplenia and is considered the most frequent form, occurring approximately in 1 in 10,000 to 40,000 births.
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Mortari et al. (2016) conducted a review in Heterotaxy syndrome. Heterotaxy syndrome with right atrial isomerism is associated with primary asplenia in over 50% of cases and has a worse prognosis than left atrial isomerism.
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