Discordant criss cross AV connection, VSD and double outlet right ventricle (DORV)
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A heart with a criss-cross atrioventricular (AV) connection is a cardiopathy in which the ventricles are positioned contralaterally to the atria to which they are connected; the ventricular inflow tracts are crossed, hence the name criss-cross.
In 1961 various cases of AV discordance with or without ventriculoarterial (VA) discordance were described. One of those cases presented as situs solitus with dextrorotation of the cardiac loop, which could correspond to the description of this abnormality.
In 1973, for the first time, in Mexico a report was published of a 4-month-old boy with situs solitus with AV concordance, VA discordance, and crossing of the ventricular inflow chambers, with anterosuperior right ventricle (RV) and posterior and inferior left ventricle (LV), ventricular septal defect (VSD), and pulmonary stenosis (PS).
These cases were later given the name of criss-cross. In the later years, other cases were described:
37 with situs solitus, 2 with situs inversus, and 1 with isomerism, most with AV concordance.
The most common VA connection was discordant transposition of the great arteries (TGA), followed by double outlet RV (DORV), associated with PS and even more rarely with pulmonary atresia.
The most common is atrial situs solitus presentation with right atrium (RA) connected to left-sided RV.