Total anomalous pulmonary venous return - supracardiac

Case contributed by Praveen Jha
Diagnosis certain

Presentation

History of shortness of breath.

Patient Data

Age: 12 years
Gender: Male

Topograph demonstrates a snowman appearance.

Both pulmonary veins are draining directly into a vertical vein via a common chamber, and ultimately draining into SVC.

Case Discussion

Anomalous drainage of pulmonary veins is seen, with right and left pulmonary veins draining into a common chamber, draining into the vertical vein, joining the innominate vein, and ultimately draining into a dilated superior vena cava.

As it is a supracardiac total anomalous pulmonary venous connection, a large atrial septal defect can be seen for draining into left atrium. No direct connection between pulmonary veins and left atrium is seen.

Diagnosis was confirmed intra-operatively, however surgery was abandoned due to the inherent risk of ligating the markedly dilated innominate vein.

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