Abstract
BACKGROUND: Malformation of the right atrium is a rare cardiac abnormality and is usually reported as isolated malformation in the literature. CASE PRESENTATION: Prenatal giant atrial dilatation in an asymptomatic infant was treated surgically at 18 months of age, due to potential risk of thrombosis and arrhythmias. Post-surgical echocardiographic images illustrate residual atrial elevated pressure. CONCLUSIONS: Sometimes, as seems in our case, right atrial dilatation hides an associated restrictive right ventricle.