Total anomalous pulmonary venous return

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Heart, section through the middle

Totally anomalous pulmonary venous return, X-ray

Totally anomalous pulmonary venous return, X-ray

Totally anomalous pulmonary venous return, X-ray

Total anomalous pulmonary venous return is a congenital heart disease (present at birth) in which none of the four veins that drain blood from the lungs to the heart is attached to the left atrium (upper chamber of the heart).

TAPVR

The cause of total anomalous pulmonary venous return (TAPVR) is unknown.

In normal circulation, blood picks up oxygen in the lungs and then returns to the left atrium. It then flows to the left ventricle, through the aorta, and around the body. In TAPVR, oxygenated blood returns to the right atrium instead. From there it goes to the right ventricle, through the pulmonary artery and back to the lungs. In other words, blood simply circles to and from the lungs and never gets out to the body.

If the infant is to live, a large atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and rest of the body.

The severity of this condition depends on whether the pulmonary veins are obstructed. There are four subtypes of TAPVR, based on the location of the veins' connection back to the heart. In one type, the veins run into the abdomen, passing through a muscle (diaphragm). This muscle squeezes the veins and narrows them, causing the blood to back up into the lungs. This type causes symptoms early in life.

Review Date: 06/27/2006
Reviewed By: Anne J. L. Chun, M.D., Assistant Professor of Pediatrics, Division of Pediatric Cardiology, New York University School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.