Morphological dilemma: Anomalous pulmonary venous confluence or cor triatriatum—does it matter?

Abstract

Cardiac variant of total anomalous pulmonary venous return is a rare entity, whereby all the pulmonary veins drain directly to the right atrium or coronary sinus. The effective left heart blood flow channels through a small stretched patent foramen ovale and can often be confused with a variant of cor triatriatum. Cor triatriatum is a rare congenital cardiac anomaly where pulmonary veins drain to a persistent chamber above the left atrium with a membrane separating these two. There persists either a small aperture directly from the true to the accessory left atrium or none at all. Where there is no such aperture, it is often physiologically akin to the cardiac variant of total anomalous pulmonary venous return described above. Such morphological differentiation is often challenging in a clinical situation, but the effective treatment remains the same. It involves removal of the common wall between the two chambers and baffling the pulmonary veins effectively to the left atrium. We describe such a case where the pulmonary venous return is to the right atrium, managed recently in our centre, and discuss the morphological differences between these two.