Isolated drainage of right superior vena cava into left atrium: A rare etiology of childhood central cyanosis.

Isolated right superior vena cava drainage into the left atrium (RSVC-LA) is an extremely rare anomaly in the absence of other cardiac or extracardiac abnormalities. The clinical and echocardiographic diagnosis is considered challenging. We report a female infant with cyanosis on crying who was diagnosed as isolated RSVC-LA using conventional and contrast echocardiography, but further confirmation was possible via multidetector CT. The surgical correction was performed with smooth postoperative recovery. In conclusion, isolated RSVC-LA must be in the differential diagnosis of cyanosis in the absence of intracardiac anomalies. Surgical correction is necessary management to guard against brain abscesses or paradoxical embolization.