Total anomalous pulmonary venous connection is a rare condition in which the pulmonary veins are connected to the heart’s upper-right chamber (the right atrium) instead of its upper-left chamber (the left atrium).

In the right atrium, the oxygen-rich blood mixes with the oxygen-poor blood entering the heart from the body. From the right atrium, this mixture of oxygen-rich and oxygen-poor blood flows through a hole called an atrial septal defect (ASD) and into the left atrium. The left atrium sends blood to the left ventricle, which pumps blood into the body.

When oxygen-poor blood is pumped into the body along with oxygen-rich blood, the fingers, toes, and lips may appear blue, a condition called cyanosis.

How is it treated?

Surgery is used to connect the pulmonary veins to the left atrium and to close the atrial septal defect. When surgery is done early enough, the long-term outlook is very good. Still, the child will need lifelong follow-up to make sure that a blockage does not develop in the pulmonary veins. There is also an increased risk for an abnormal heart rhythm (called an arrhythmia).