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Total anomalous pulmonary venous return (TAPVR)

Medically reviewed by Drugs.com. Last updated on Jan 4, 2023.

Overview

Total anomalous pulmonary venous return (TAPVR) is a rare heart problem that's present at birth. That means it's a congenital heart defect.

Other names for this condition are:

  • Total anomalous pulmonary venous connection
  • TAPVC

In this heart condition, the lung blood vessels, called the pulmonary veins, attach to the wrong place in the heart.

In a typical heart, oxygen-rich blood flows from the lungs to the upper left heart chamber, called the left atrium. The blood then flows through the body.

In TAPVR, the connection of veins is changed. Blood flows through the upper right heart chamber, called the right atrium. This change in blood flow causes oxygen-poor blood to mix with oxygen-rich blood. As a result, blood flowing to the body doesn't have enough oxygen.

The specific type of TAPVR depends on where the veins connect. Most children born with TAPVR have no family history of congenital heart disease.

Total anomalous pulmonary venous return

In total anomalous pulmonary venous return (TAPVR), the pulmonary veins incorrectly send blood to the heart's upper right chamber. That chamber is called the right atrium. As a result, oxygen-rich blood mixes with oxygen-poor blood, as shown in purple. In a typical heart, shown on the left, oxygen-rich blood flows from the pulmonary veins to the upper left chamber, also called the left atrium.

Symptoms

A baby with TAPVR may have breathing difficulty. The baby's skin may look gray or blue due to low oxygen levels. This is called cyanosis.

A health care provider may notice symptoms of TAPVR soon after birth. But some children don't have symptoms until later on.

Diagnosis

Your child's health care provider does a physical exam and listens to your child's heart with a stethoscope. A whooshing sound, called a heart murmur, may be heard.

An echocardiogram is the test used to diagnose total anomalous pulmonary venous return. This test uses sound waves to create images of the heart in motion. An echocardiogram can show the pulmonary veins, any holes in the heart and the size of the heart chambers. It also shows blood flow through the heart and heart valves.

Other tests such as an electrocardiogram, a chest X-ray or a CT scan may be done if more information is needed.

Treatment

Surgery for TAPVR is generally needed when a child is a baby. The timing of surgery depends on whether there's a blockage. To repair the heart, surgeons connect the pulmonary veins to the left upper heart chamber. They also close the hole between the upper heart chambers.

A person with total anomalous pulmonary venous return needs regular health checkups for life to check for infection, blockages or heart rhythm problems. A doctor trained in congenital heart diseases should provide care. This type of provider is called a congenital cardiologist.

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