Totally Anomalous Pulmonary Venous Return, Supradiaphragmatic (TAPVR)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Etiology/Pathophysiology:
Instead of empyting into the left atrium, the pulmonary veins empty into the right atrium.

In TAPVR the blood can go from the retrocardiac common pulmonary vein to 3 places:

• Supracardiac into the left vertical vein (persistant left superior vena cava) which drains into the left brachiocephalic vein which drains into the superior vena cava. This is the most common form of anomalous pulmonary venous return (80-90% ).
• Cardiac into the coronary sinus or right atrium
• Infradiaphragmatic into a vertical vein that descends through the diaphragm into the portal vein and or inferior vena cava.

Supracardiac TAPVR rarely has obstruction and is associated with an obligatory right to left shunt at the atrial level via a patent foramen ovale or ASD.

Pathology:
Not applicable

Imaging Findings:
Significant shunt vascularity and features resembling a large ASD are normally seen. In older children a"snowman" configuration to the mediastinum is due to the vertical vein and the dilated superior vena cava.

DDX:

• Not applicable

References:
See References Chapter.

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See related Provider Topics Congenital Heart Disease, Genetics/Birth Defects or Heart and Circulation.

See related Patient Topics Congenital Heart Disease, Genetics/Birth Defects or Heart and Circulation.

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