Total Anomalous Pulmonary Venous Return

Pulmonary veins normally bring oxygenated blood back from the lungs to the left atrium. In total anomalous pulmonary venous connection all the pulmonary veins drain into the right atrium. In order for the infant to survive, they must have some communication between the right and left sides of the heart to allow oxygenated blood to reach the body. This is always an ASD, although a VSD may be present as well. Because this oxygenated blood is so dilute, the infant may appear blue or "cyanotic".

There are three main types of TAPVR, depending on where the pulmonary veins drain. There are referred to as supracardiac, intracardiac, and infracardiac. There may also be a mixed type, in which two or more types may coexist.

Open heart surgery is needed in early infancy. The surgery involves removal of the pulmonary veins from the right atrium and anastomosis to the left atrium. The ASD is also closed, along with the division of any abnormal connections that may be present.

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