Total Anomalous Pulmonary Venous Return (TAPVR)

Total anomalous pulmonary venous return is a heart condition in which blood vessels that are supposed to carry blood from the lungs to the left side of the heart go to the right side instead. This condition is present at birth (congenital heart defect).

In a normal heart, blood from all over the body returns to the right side of the heart. The right side of the heart pumps blood to the lungs, where the blood picks up oxygen. The oxygen-rich blood then flows to the left side of the heart through the blood vessels (pulmonary veins) and back out to the body.

If your child has TAPVR, the pulmonary veins return all the oxygen-rich blood to the right side of your child’s heart instead. The oxygen-rich blood mixes with the returning oxygen-poor blood and passes through a hole between the right and left sides of the heart (atrial septal defect). This makes the heart work harder, which results in less oxygen-rich blood going out to the body. The symptoms of TAPVR usually show up soon after birth, but sometimes they start later.

What are the causes?

The cause of this condition is not known.

What are the signs or symptoms?

Symptoms of this condition can vary from mild to severe. They include:

• Abnormal heart sound (murmur).
• Pounding heart.
• Weak pulse.
• Blue lips, skin, or fingers caused by a lack of oxygen (cyanosis).
• Rapid and difficult breathing.
• Poor feeding.
• Sleepiness and lack of alertness (lethargy).

How is this diagnosed?

Before birth, this condition may be diagnosed with:

• Routine imaging tests that use sound waves to create images of your baby (fetal ultrasound).
• Tests that use sound waves to create images of your baby’s heart (fetal echocardiogram).

After birth, this condition may be diagnosed based on:

• Your child’s symptoms.
• A physical exam. Your child’s health care provider may listen to the heart to check for a murmur.
• Chest X-ray to check for an enlarged heart and fluid in the lungs.
• Echocardiogram to check for abnormal pulmonary veins.
• Cardiac catheterization. During this procedure, a flexible tube (catheter) is inserted into a blood vessel and guided into the heart. This measures pressures and checks for an atrial septal defect.
• Pulse oximetry. This measures the oxygen level of your child’s blood.
• Electrocardiogram (ECG). This tests the electrical activity of your child’s heart. This may show that the left side of the heart is getting larger from working too hard.

How is this treated?

This condition is treated with open-heart surgery to connect the pulmonary veins to the left side of the heart and close the atrial septal defect. If your baby has very serious symptoms, surgery is usually done as soon as possible.

Summary

• Total anomalous pulmonary venous return (TAPVR) is a heart condition in which blood vessels that are supposed to carry blood from the lungs to the left side of the heart go to the right side instead. This condition is present at birth (congenital heart defect).
• Before birth, this condition may be diagnosed with ultrasound. After birth, it may be diagnosed by using your child’s symptoms, imaging tests, as well as pulse oximetry and ECG.
• This condition is treated with open-heart surgery to connect the pulmonary veins to the left side of the heart and close the atrial septal defect. If your baby has very serious symptoms, surgery is usually done as soon as possible.