A Rare Form of Supracardiac Total Anomalous Pulmonary Venous Drainage—Evaluation by Computed Tomography and Magnetic Resonance Imaging

Abstract

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon congenital cardiac anomaly. It constitutes about 2– 5% of all congenital cardiac diseases at autopsy [1,2]. In TAPVD, the pulmonary veins may drain aberrantly into any tributary of the sinus venosus. There are four main patterns of drainage: supracardiac, cardiac, infradiaphragmatic, and mixed. Echocardiography is a useful screening technique for demonstrating the presence and site of anomalous drainage, including assessment of obstruction and associated intracardiac anomalies [3–5]. However, it is limited in its ability to demonstrate the draining vein if this is located away from the chest wall. Angiocardiography still remains the standard investigation [6]. However, this technique is invasive and has a significant risk of complications, especially in cyanosed neonates and infants. The role of computed tomography (CT) and magnetic resonance imaging (MRI) for evaluation of TAPVD has been well described in the literature [6,8–10]. Both these techniques are capable of demonstrating the anomalous venous connection, including the evaluation of obstruction, at any site and in any desired plane. We describe the CT and MRI features in a very rare variant of supracardiac TAPVD, with an intrapulmonary location of the common venous channel. Only 10 such cases have been reported so far, five of whom had normal cardiac anatomy (as in the case presented) [7]. To our knowledge, the CT and MRI findings in this form of drainage have not been described previously.