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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 take blood from the lungs to the heart is attached to the left atrium (left upper chamber of the heart).

Alternative Names

TAPVR

Causes, incidence, and risk factors

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

In normal circulation, blood is sent from the right ventricle to pick up oxygen in the lungs. It then returns through the pulmonary veins to the left side of the heart, which sends blood out through the aorta, and around the body.

In TAPVR, oxygenated blood returns from the lungs back to the right atrium or right ventricle -- instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body.

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 rest of the body.

The severity of this condition depends on whether the pulmonary veins are obstructed. There are four subtypes of TAPVR, based on the location of the veins' connection back to the heart. In one type, the veins run 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.

Symptoms

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

Note: Sometimes, no symptoms may be present in infancy or early childhood.

Signs and tests

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

Treatment

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

Expectations (prognosis)

If left untreated, death may occur by age 1 in babies with more severe defects. With surgery, early repair provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart.

Complications

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.

References

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.

Review Date:12/10/2007
Reviewed By:David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.; and Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of Philadelphia.

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