[Skip to Content]

A to Z: Total Anomalous Pulmonary Venous Return

A to Z: Total Anomalous Pulmonary Venous Return

May also be called: TAPVR, Total Veins, Total Anomalous Pulmonary Venous Connection, TAPVC

Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect where the pulmonary veins don’t connect to the left atrium the way they normally would. "Congenital" means a person is born with it. TAPVR causes blood coming from the lungs to flow into the wrong part of the heart.

More to Know

After arriving in the right ventricle of the heart, blood that's low in oxygen is pumped to the lungs to pick up more oxygen. The blood, now full of oxygen, flows back from the lungs to the left atrium of the heart through the four pulmonary veins. But sometimes these veins don't attach to the left atrium when a baby is developing in the womb. Instead, they connect to other blood vessels or a different part of the heart. If all four pulmonary veins connect to somewhere other than the left atrium, the condition is called total anomalous pulmonary venous return, or TAPVR.

TAPVR causes blood from the lungs to go back to the right side of the heart instead of the left side. From the right side, the blood is sent back to the lungs and may not get pumped out to the body at all. In cases where blood is being pumped out to the body, it’s a mixture of blood full of oxygen and blood low in oxygen. Low oxygen can cause breathing problems and make the skin a different color (depending on the child's normal skin color, it can look blue, green-ish, or around the gums and eyes, gray/white). 

There are different types of TAPVR based on where the pulmonary veins connect. Doctors don’t know what causes TAPVR, but it can usually be detected during pregnancy and treated shortly after birth. Treatment for TAPVR involves open-heart surgery to put the pulmonary veins in the correct place.

Keep in Mind

If TAPVR is detected before or just after birth and corrected through surgery, the long-term outlook is very good. If it goes untreated, TAPVR will lead to heart failure. Children who have surgery to correct TAPVR will have to follow up regularly with a cardiologist. Most kids will have no limits on their activity.

All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.