Totally Anomalous Pulmonary Venous Return, Infradiaphragmatic (TAPVR)

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

Etiology/Pathophysiology:
The pulmonary venous drainage goes from the common pulmonary vein to the vertical retrocardiac pulmonary vein which goes through the esophageal hiatus to empty into the ductus venosus / portal vein / tributary of the portal vein. This causes a relative obstruction to pulmonary venous drainage and results in pulmonary venous hypertension. There is diminished blood flow to the left side of heart and to the right side of the heart so there is no volume overload and no cardiac enlargement.

Pathology:
Not applicable

Imaging Findings:
On CXR there is obvious pulmonary venous hypertension and edema, but no cardiomegaly. An esophagram can show an anterior impression on a barium filled esophagus by a retrocardiac common pulmon vein.

DDX:
Pulmonary venous hypertension with cardiomegaly

• Hypoplastic left heart
• Critical aortic stenosis
• Cor triatriatum
• Pulmonary venous atresia

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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