Total Anomalous Pulmonary Venous Connection (tapvc)

Jagdish Kathwate
MD Pediatrics. Assistant Professor, Government Medical College, Aurangabad, India.
First Created: 01/09/2002  Last Updated: 08/01/2015

Patient Education

What is it TAPVC?

A defect in the veins leading from the lungs to the heart. In TAPVC, the blood does not take the normal route from the lungs to the heart and out to the body. Instead, the veins from the lungs attach to the heart in abnormal positions and this problem means that oxygenated blood enters or leaks into the wrong chamber.

What causes it?

In most children, the cause isn't known. Some children can have other heart defects along with TAPVC.

How does it affect the heart?

In the right atrium, oxygen-rich blood from the pulmonary veins mixes with low-oxygen blood from the body. Part of this mixture passes through the atrial septum into the left atrium. From there it goes into the left ventricle, then into the aorta and out to the body. The rest of the blood flows through the right ventricle, into the pulmonary artery and on to the lungs. The blood passing through the aorta to the body doesn't have a normal amount of oxygen, which causes the child to look blue.

How does TAPVC affect a child?

Symptoms may develop soon after birth. In other children, symptoms may be delayed. This partly depends on whether the lung veins are blocked as they drain toward the right atrium. Severe obstruction of the pulmonary veins tends to make infants breathe harder and look bluer (have lower oxygen levels) than infants with little obstruction.

What can be done about the defect?

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.

What will the child need in the future?

When surgical repair is done in early infancy, the long-term outlook is very good. However, the child will need regular follow-up with a pediatric cardiologist and, once the child reaches adulthood, lifelong regular follow-up with a cardiologist who's had special training in congenital heart defects. Follow-up is needed to make certain that any remaining problems, such as an obstruction in the pulmonary veins or irregularities in heart rhythm, are treated. Some children may need medicines, heart catheterization, or even more surgery.

What about preventing endocarditis?

Children with TAPVC are at increased risk of developing endocarditis. The child will need to take antibiotics before certain dental procedures to help prevent endocarditis.

Total Anomalous Pulmonary Venous Connection (TAPVC) Total Anomalous Pulmonary Venous Connection (TAPVC) 2015-08-01
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