Total Anomalous Pulmonary Venous Drainage

In the normal human heart, oxygen-rich blood returns from the lungs through four pulmonary veins to the left atrium.

In total anomalous pulmonary venous drainage (TAPVD), the pulmonary veins (4) instead drain through abnormal connections (3) to the right atrium. This means that oxygen-rich blood mixes with oxygen-poor blood. Some of this mixture passes from the right atrium to the left atrium (through an atrial septal defect—ASD, 2) and then through the left ventricle, aorta, and on to the body.

Since this blood does not have enough oxygen, the baby may look blue. Symptoms (congestive heart failure, respiratory infections) can develop soon after birth. This defect must be surgically repaired in early infancy.

In surgery, the pulmonary veins are reconnected to the left atrium and the ASD is closed. Lifelong follow-up is needed to make certain that any other problems such as blockage of the pulmonary veins or irregularities in heart rhythm are treated properly.