Cor triloculare biventriculare with left superior vena cava

Cor triloculare biventriculare is a rare congenital malformation of the heart in which there is a complete absence of the atrial septum. It is usually associated with other anomalies like complete atrioventricular canal defect, polysplenic syndrome, isolated dextrocardia, Ellis-van Creveld syndrome, or persistent left superior vena cava. We report a case of a stillborn male foetus of 35 weeks gestation with common atrium, complete atrioventricular canal defect, and persistent left superior vena cava. The possible embryological basis and clinical implication of this variation are discussed.