DOI: 10.36347/sjmcr.2021.v09i11.013

Pages: 1090-1092

The detection of rare congenital cardiac anomalies gives us an opportunity to study such infrequent conditions in greater detail. In this report we describe such an entity that has been reported in the literature extremely rarely. A six weeks old baby boy presented with respiratory distress with features of cardiac failure. The echocardiogram revealed a Criss cross heart with atrioventricular segmental discordance, with a large ventricular septal defect, atrial septal defect and pulmonary arterial hypertension. The ventricles did not have the expected supero-inferior position. There was no ventriculoarterial discordance with no atrioventricular valve straddling. This condition is a rare congenital anomaly. Absence of supero-inferior position of the ventricles, ventriculoarterial discordance and straddling of atrioventricular valves make this case most unusual. Hence such variations need to be considered while entertaining a diagnosis of Criss cross heart in future reports.