Terminal 6q deletion syndrome is an infrequent disorder with dearth of data in the literature. Some reports of chromosome 6q deletion showed an association with congenital heart diseases such as atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), Tetralogy of Fallot (TOF) and double outlet right ventricle (DORV). Persistent left superior vena cava (PLSVC) drainage into the left atrium (LA) is a rare congenital thoracic venous anomaly. In this paper. We demonstrate the first reported association of 6q deletion syndrome with PLSVC drainage into the LA.

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