Criss cross heart in a congenitally corrected transposition of the great arteries

Criss cross heart is an extremely rare anomaly accounting for less than 0.1% of congenital heart disease. It is characterized by an abnormal rotation of the heart on its long axis. Almost always there are associated cardiac anomalies such as pulmonary stenosis, ventricular septal defect, ventriculoarterial connection discord or hypoplasia of the tricuspid valve or right ventricle and thereby presenting in the neonatal period. We present a case of a 22 year old woman with a congenitally corrected transposition of great arteries and a criss cross heart.