Total Anomalous Pulmonary Venous Return (TAPVR)

What is total anomalous pulmonary venous return?

Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection (TAPVC), is a rare heart defect in which the blood vessels that drain the lungs (pulmonary veins) are not connected normally to the heart. Instead, the pulmonary veins are redirected abnormally to other chambers of the heart. About 1 in every 20,000 babies is born with TAPVR.

In order to get blood to the body, most babies with TAPVR also have another heart defect, called atrial septal defect, which is a hole from the right atrium to the left atrium.

There are four major types of TAPVR. Each is based on where the pulmonary veins connect to the heart:

  • supracardiac, where the pulmonary veins make an abnormal connection above the heart
  • cardiac, where the pulmonary veins connect behind the heart
  • infracardiac, where the pulmonary veins connect below the heart
  • mixed, a combination of any of the connections above

TAPVR can occur with obstruction, meaning that some of the draining blood vessels are obstructed. This can cause high blood pressure in the lungs (pulmonary hypertension) and can be a surgical emergency.

Children with TAPVR will need surgery in infancy to repair the problem.

How we care for total anomalous pulmonary venous return

Our team in the Boston Children’s Department of Cardiac Surgery treat some of the most complex pediatric heart conditions in the world. Our specialized clinicians can often diagnose this condition during fetal echocardiogram.

Our cardiac surgeons have vast experience in repairing this defect, and work with nurses and doctors who are focused on providing expert care after surgery. Adult patients with TAPVR are followed by Boston Children’s cardiologists with special training for adults with congenital heart problems.