TAPVR

Alternative names
Total anomalous pulmonary venous return

Definition
Total anomalous pulmonary venous return is a congenital heart disease (present at birth) in which none of the four veins that drain blood from the lungs to the heart is attached to the left atrium (upper chamber of the heart).

Causes, incidence, and risk factors

The cause of total anomalous pulmonary venous return (TAPVR) is unknown.

In normal circulation, blood is oxygenated in the lungs and then returns to the left atrium, flowing from there to the left ventricle, through the aorta, and around the body. In TAPVR, oxygenated blood returns to the right atrium instead. From there it goes to the right ventricle, through the pulmonary artery and back to the lungs. In other words, blood simply circles to and from the lungs and never gets out to the body. This condition is incompatible with life.

If the infant is to live, a large atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and subsequently the body.

The severity of this condition depends on whether the pulmonary veins are obtructed. There are two subtypes of TAPVR. In one, the veins course into the abdomen, passing through a muscle (diaphragm). This muscle squeezes the veins and narrows them, causing the blood to back up into the lungs. This type causes symptoms early in life.

The second type of TAPVR has no obstruction; the veins do not course through the abdomen.

Symptoms

The infant may appear to be critically ill and may display the following symptoms:

Note: In some cases no symptoms may be present in infancy or early childhood.

Signs and tests

     
  • An ECG shows signs of enlargement of the ventricles (ventricular hypertrophy).  
  • An X-ray of the chest shows a normal to small heart with fluid in the lungs.  
  • An echocardiogram usually defines the attachment of pulmonary vessels.  
  • Cardiac catheterization can provide definitive diagnosis by showing abnormal attachments of the blood vessels.

Treatment
Early complete surgical repair is indicated. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.

Expectations (prognosis)
Early repair appears to give excellent results, provided there are no additional heart abnormalities.

Complications

     
  • heart failure  
  • breathing difficulties  
  • lung infections

Calling your health care provider

This condition may be apparent at the time of birth. However, symptoms may not be present until later.

Call your health care provider if you notice symptoms of TAPVR - prompt attention is required!

Prevention

There is no known way to prevent TAPVR.

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

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