Total anomalous pulmonary venous return (TAPVR)
Medically reviewed by Drugs.com. Last updated on Jun 23, 2021.
Total anomalous pulmonary venous return (TAPVR) is a rare heart defect that's present at birth (congenital heart defect). It is sometimes called total anomalous pulmonary venous connection (TAPVC).
In this heart defect, the lung blood vessels (pulmonary veins) are attached to the wrong place in the heart.
Normally, oxygen-rich blood goes from the lungs to the upper left heart chamber (left atrium) and then flows through the body. In TAPVR, an abnormal connection of veins sends blood through the upper right heart chamber (right atrium) instead, where it mixes with oxygen-poor 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.
In total anomalous pulmonary venous return (TAPVR), the pulmonary veins incorrectly send blood to the heart's upper right chamber (atrium). In a normal heart (shown on the left), oxygen-rich blood flows from the pulmonary veins to the upper left chamber (left atrium). In a type of TAPVR called supracardiac TAPVR, oxygen-rich blood from the pulmonary veins goes into the wrong chamber on the other side (right atrium). As a result, the blood mixes with oxygen-poor blood.
A baby with TAPVR may have breathing difficulty and appear blue (cyanotic) because of the lack of oxygen in the blood that goes out to the body, or because blood flow through the pulmonary veins is reduced or blocked.
A doctor may notice signs and symptoms of TAPVR soon after birth. But some children don't have symptoms until later on.
Your child's doctor will perform a physical exam and listen to your child's heart with a stethoscope to check for a heart murmur.
An echocardiogram is generally used to diagnose total anomalous pulmonary venous return. This test uses sound waves to create images of your child's heart in motion. An echocardiogram can show pulmonary veins, holes in the heart and the size of the heart chambers. It can also measure the rate of blood flow.
Other tests such as an electrocardiogram (ECG or EKG), a chest X-ray or computerized tomography (CT) scan may be done if more information is needed.
Surgery is generally needed when a child is a baby. The timing of surgery depends on whether or not there's a blockage. To repair this congenital heart defect, surgeons connect the pulmonary veins to the left atrium and close the hole between the atria.
A person with total anomalous pulmonary venous return will need regular doctor's checkups with cardiologists trained in congenital heart disease to monitor for infection, blockages or heart rhythm problems.