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Total Anomalous Pulmonary Venous Connection (TAPVC)

TAPVC is a defect in the veins where the blood does not take the normal route from the lungs to the heart and out to the body. Instead, the veins coming from the lungs attach to the heart in abnormal positions.

This means that oxygenated blood enters or leaks into the wrong chamber. The blood passing through the aorta to the body doesn't have enough oxygen. This causes you or, more likely, your child to look blue.

What happens once you have TAPVC?

Symptoms of TAPVC may develop soon after birth or may come later. This depends on whether the lung veins are blocked as they drain toward the right atrium.

Infants with TAPVC can have symptoms like:

  • Weak pulse.

  • Poor feeding.

  • Pounding heart.

  • Problems breathing.

  • Extreme sleepiness.

  • Ashen or bluish skin color.

It's rare for an adult to have uncorrected TAPVC. Patients who survive until adulthood usually have only a mild bluish color (cyanosis) and no blockage of their arteries. Symptoms include shortness of breath and inability to exercise.

What causes TAPVC?

The causes of heart defects such as TAPVC are unknown. Some babies have heart defects because of changes in their genes or chromosomes.

Heart defects may also be caused by a combination of genes and other risk factors, like the environment, what the mother eats or drinks, or the medicines she uses.

What types of tests are used to diagnosis TAPVC?

TAPVC might be diagnosed during pregnancy with an ultrasound or a fetal echocardiogram. However, it is easier to detect this defect after birth when the blood flows to the lungs and returns to the heart.

Using a stethoscope, a doctor may hear a heart murmur (an abnormal “whooshing” sound). If a doctor thinks there might be a problem, the doctor may want to use any of these tests:

  • Echocardiogram: This ultrasound can show problems with the structure of the heart.

  • Cardiac catheterization: With a camera, this catheter can show if any blood vessels are abnormally attached.

  • Electrocardiogram (EKG): This test measures the electrical activity of the heart.

  • Chest X-rays: These images may be used to find any abnormalities.

  • Newborn pulse oximetry screening: Pulse oximetry can determine the amount of oxygen in a baby’s blood.

What types of treatments and procedures are used to treat TAPVC?

This defect must be surgically repaired in early infancy. At the time of open-heart surgery, the pulmonary veins are reconnected to the left atrium, and the atrial septal defect is closed.

The age at which the surgery is done depends on how sick the child is and the specific structure of the abnormal connections between the pulmonary veins and the heart. The goal of the surgery is to restore normal blood flow.

To repair this defect, doctors usually connect the pulmonary veins to the left atrium and close any abnormal connections between blood vessels.

What can I do to support my health when I have TAPVC?

Patients with a history of TAPVC should be seen periodically by a cardiologist with expertise in adult congenital heart disease to look for uncommon problems. The long-term outlook is excellent.

Your cardiologist might monitor you with noninvasive tests if needed. These include:

  • Holter monitors.

  • Electrocardiograms.

  • Exercise stress tests.

  • Echocardiograms.

Most adults with repaired TAPVC will not need to limit their physical activities. Some competitive sports may have a greater risk if there is a leftover obstruction in the pulmonary veins or if a patient has heart rhythm problems. Your cardiologist can help you decide how much physical activity you can do.

Why choose Presbyterian for TAPVC treatment?

Presbyterian’s Pediatric and Congenital Cardiology team has many different options to help you manage your or your child’s heart condition. The team performs various diagnostic tests and procedures to help form an accurate diagnosis and create individualized treatment plans. Depending on the type of heart condition your child has and its underlying cause, the team can recommend a wide variety of treatment options. Our pediatric cardiologists, pediatric interventional cardiologists, and pediatric cardiovascular surgeons work closely together for cases in which cardiac repair or surgery is the best treatment option.