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Virtual Pediatric Hospital: Paediapaedia: Totally Anomalous Pulmonary Venous Return, Supradiaphragmatic (TAPVR) Paediapaedia: Cardiovascular Diseases

Totally Anomalous Pulmonary Venous Return, Supradiaphragmatic (TAPVR)

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


Clinical Presentation:
The cyanosis is mild due to a large volume of blood being continuously circulated throughout the lung. Present with a large left to right shunt with congestive heart failure.

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 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:

References:
See References Chapter.

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