Total anomalous pulmonary venous drainage (TAPVD)

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Learn about total anomalous pulmonary venous drainage (TAPVD), a condition involving improper drainage through the blood vessels. TAPVD can be fatal.

Key points

  • Children with total anomalous pulmonary venous drainage are usually cyanotic, have trouble breathing, and have a heart murmur.
  • Surgery is required very shortly after birth, allowing for a very good outlook for children.

In total anomalous pulmonary venous drainage (TAPVD), the vessels that bring oxygen-rich blood back to the heart from the lungs are incorrectly connected to the left atrium. Blood drains to the right atrium through an abnormal vessel.

The two images below compare a normal heart to

Normal heart Normal heart showing placement of the atria, ventricles, aorta, superior vena cava, inferior vena cava, pulmonary artery and pulmonary veins
The heart has two upper (receiving) and two lower (pumping) chambers. Blood flows into the upper chambers (the right atrium and the left atrium). The lower chambers (the right and left ventricles) pump blood out of the heart. The heart valves open and close to keep blood flowing in the correct direction.
Total anomalous pulmonary venous drainage Heart with pulmonary veins attached incorrectly to superior vena cava through a vertical vein. Atrial septal defect allows blood high in oxygen to reach left ventricle.
TAPVD is a condition in which all four of the pulmonary veins are abnormally connected to the right atrium instead of the left atrium. This allows blood high in oxygen to enter the right side of the heart. Blood high in oxygen flows across an ASD, to the left ventricle, allowing some blood with oxygen to reach the body. Without the ASD, blood high in oxygen would not be able to reach the body.

An atrial septal defect (ASD) usually accompanies this condition, allowing blood to flow from the right atrium to the left atrium. Without an ASD, the condition is fatal because there is no oxygenated blood returning from the heart to be circulated to the body.

An atrial septal defect (ASD) usually accompanies this condition, allowing blood to flow from the right atrium to the left atrium. Without an ASD, the condition is fatal because there is no oxygenated blood returning from the heart to be circulated to the body.

What are the symptoms of total anomalous pulmonary venous drainage?

Children with total anomalous pulmonary venous return are usually cyanotic, have trouble breathing, and have a heart murmur.

Chest X-rays, echocardiograms, or a cardiac catheterization may be done to diagnose the condition.

How is total anomalous pulmonary venous drainage treated?

Surgery is done very shortly after birth to improve this condition. This involves connecting the pulmonary veins to the left atrium and closing the ASD.

What is the long-term outlook for children with total anomalous pulmonary venous drainage?

With early repair, the outlook is very good for children with this condition.

Last updated: December 4th 2009