Atrial Isomerism




Abstract


Atrial isomerism is a very rare condition defined as symmetric development of normally asymmetric organs or organ systems. The combination of situs ambiguus and congenital heart disease is the main clue for the diagnosis. It associates poor prognosis due to its high in utero and neonatal mortality.




Keywords

isomerism, heterotaxy, situs ambiguus, cardiosplenic syndrome

 




Introduction


Heterotaxy (or situs ambiguus ) is defined as the abnormal arrangement of thoracic or abdominal organs, or both, across the left-right axis ( ); it is different from complete situs inversus, which involves all organs. The term isomerism refers to the symmetric development of normally asymmetric organs or organ systems; this is the main feature of heterotaxy syndromes. These syndromes comprise a combination of cardiac, vascular, and visceral abnormalities. The terms heterotaxy syndrome, cardiosplenic syndrome, right and left isomerism, and situs ambiguus are often used to describe these defects.


Atrial isomerism manifests as a varied spectrum of corporeal and cardiac malformations. It is associated with a very poor prognosis owing to high in utero and neonatal mortality.




Disease


Definition


Isomerism is defined as symmetric development of normally asymmetric organs or organ systems. The combination of situs ambiguus and congenital heart disease is the main clue for the diagnosis.


Prevalence and Epidemiology


Atrial isomerism is very rare, accounting for 4% of congenital heart anomalies.


Etiology and Pathophysiology


Atrial isomerism manifests with a varied spectrum of cardiac malformations with viscerocardiac heterotaxy occurring in most cases. Although there is wide variation in the range of anomalies that may be present, isomerism is classified as follows.


Jul 7, 2019 | Posted by in OBSTETRICS & GYNAECOLOGY IMAGING | Comments Off on Atrial Isomerism
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