Topsy-Turvy Heart: A Very Rare Case of Superoinferior Ventricle

Abstract

This report presents computed cardiac tomographic (CT) and echocardiographic images of a 20-day-old newborn with a suspected cardiac rotation abnormality in the setting of levocardia, atrial situs solitus, atrioventricular concordance, superoinferior orientation of the ventricles, and ventriculoarterial concordance.

Mutations in GREB1L Cause Bilateral Kidney Agenesis in Humans and Mice

“…This phenotype is reminiscent of topsy-turvy heart. 35 Despite misorientation of these structures, the atria and the great vessels were seemingly well formed. All the other organs (lung, liver, intestine, stomach) appear to be normal and well positioned, although slightly underdeveloped.…”

Concordant Atrioventricular Connection to L-Looped Ventricles with the Left Ventricle on Top of the Right Ventricle in Situs Solitus: A Case Report with 3D Modelling and Printing

“…The disharmonious connection was associated with the unusually elongated left atrium with its appendage juxtaposed on top of the right atrial appendage, and forward, superior and rightward displacement of the mitral valve annulus. The ventricles are superoinferiorly related and the four chambers of the heart were oriented in the coronal plane instead of the axial one, which is similar to that described in so-called topsy-turvy hearts [6][7][8]. In contrast to most hearts with superoinferior relationship of the ventricles including topsy-turvy hearts in which the right ventricle is the superior ventricle, the current case had the left ventricle on top of the right ventricle.…”

Topsy-Turvy Heart with Aortopulmonary Window and Severe Airway Malacia: Prenatal Diagnosis and Review of the Literature

“…Our patient also expired after palliative care and agreement with the family. There were no data about treatment provided or outcome in the rest of the seven cases reported [1,7,10].…”