Classical chest x-ray finding in TAPVC

What is the classical chest x-ray finding in TAPVC?

The classical chest x-ray finding in TAPVC (total anomalous pulmonary venous connection) is “snow man in snow storm appearance”. It is also called “figure of eight appearance” and “cottage loaf appearance”. The lower part of the figure of eight is the heart and the upper part is contributed by the vertical vein on the left and dilated superior vena cava on the right side. The dilated left brachiocephalic vein forms the superior link. The snow man in snow storm description is because the lung fields are congested, mimicking snow storm.

Classical X-Ray patterns may take some time to develop and may not be evident in very young infants. X-Ray of a typical ‘Snowman’ or ‘cottage loaf’ heart shape due to total anomalous pulmonary venous drainage has been demonstrated by J Somerville, V Grech and colleagues [1].

Though TAPVC usually presents in early infancy, later presentations are not unknown. Norain Talib reported a woman presented at 33 years after having undergone a successful pregnancy and delivery [2]. Chest X-Ray showed the typical snowman appearance. Echocardiography showed large atrial septal defect with suspicion of pulmonary venous anomaly. She underwent cardiac catheterization and CT which confirmed supracardiac variety of TAPVC. She underwent successful surgical repair.

References

1. J Somerville, V Grech. The Chest X-Ray in Congenital Heart Disease 1. Total Anomalous Pulmonary Venous Drainage and Coarctation of the Aorta. Images Paediatr Cardiol. 2009 Jan;11(1):7-9.
2. Norain Talib, Khairil Amir Sayuti, Mohd Shafie Abdullah, Mohd Rizal Mohd Zain. Beating the Odds: A Rare Case of Supracardiac Total Anomalous Pulmonary Venous Return (TAPVR) in an Adult Patient. BMJ Case Rep. 2018 Mar 5;2018:bcr2017221074.