Version July 2025
Please read the Disclaimer at the end of this page.
What is a total anomalous pulmonary venous connection? —
Total anomalous pulmonary venous connection, or "TAPVC," is a rare condition that involves the veins that carry blood from the lungs to the heart. These veins aren't connected to the correct spot in the heart. The condition is also known as "total anomalous pulmonary venous return," or "TAPVR."
Normally, blood flows through the heart and lungs in the following way (figure 1):
●Blood comes in from the body through a part of the heart called the right atrium. Then, it flows into another part of the heart, called the right ventricle.
●Next, the blood goes to the lungs, where it picks up oxygen.
●The blood comes back into the heart through the left atrium, then into the left ventricle.
●From there, the heart pumps the blood around the body.
In a TAPVC, blood from the lungs does not return to the left atrium the way it should. Instead, it returns to the right atrium. Or the blood returns to another vein in the body and then to the right atrium. As a result, the blood with the oxygen can't easily get to the organs in the body. Plus, sometimes, the veins carrying the blood from the lungs get blocked. Both of these things can cause symptoms.
There are different types of TAPVC, depending on where the veins from the lungs attach. TAPVC is a condition that children are born with. Doctors don't know what causes it.
What are the symptoms of a TAPVC? —
Symptoms depend on the type of TAPVC and whether the veins carrying blood from the lungs are blocked.
Babies whose condition is severe get very sick within the first 12 hours after birth. They don't have enough oxygen in their blood, and they have a lot of trouble breathing.
Babies whose condition is not as severe usually start having symptoms later on. These can include:
●Breathing faster than usual
●Not feeding well
●Not gaining weight or growing well
●Skin that looks blue
Will my baby need tests? —
Yes. The test most often done to check for a TAPVC is an echocardiogram, or "echo." It uses sound waves to create a picture of the heart as it beats. It shows how blood flows through the heart and lungs.
Sometimes, doctors do other imaging tests. These create pictures of the inside of the body.
How is a TAPVC treated? —
It is treated with surgery. During surgery, the doctor will fix or move the veins so that blood from the lungs gets to the correct place.
But before your child has surgery, their breathing and general medical condition need to be managed. If your baby's condition is severe, they might need 1 or more of the following before surgery:
●Help with breathing – This can include oxygen treatment or a breathing tube. A breathing tube is a tube that goes down the throat and into the lungs. The other end is attached to a machine that helps with breathing.
●Medicines that help the heart
●"Cardiac catheterization" – For this procedure, a doctor places a thin tube into a blood vessel in the baby's leg. Then, they move the tube through the blood vessel to the heart. There, the tube can be used to open up the area that is blocked. This helps the blood with oxygen get into the left atrium and out to the rest of the body.
●A heart and lung machine, called "ECMO" – This machine takes over the jobs of the heart and lungs. It pumps blood from your baby's body, gives it oxygen, and pumps it back into their body.
What will my child's life be like after treatment? —
It depends on your child's individual situation. All children with TAPVC need to see their heart doctor for regular follow-ups.
Sometimes, after surgery, a problem can happen with 1 of the veins. If this happens, a child might need surgery again. Also, children sometimes have heart rhythm problems after their surgery.
Patient education: Atrial septal defects in children (The Basics)
Patient education: Cardiac catheterization (The Basics)
Patient education: Extracorporeal membrane oxygenation (ECMO) (The Basics)
Patient education: Newborn appearance (The Basics)
