Total Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return (TAVPR) is a type of congenital heart defect (present at birth) that affects the pulmonary veins. Arteries carry blood away from the heart; veins carry it toward the heart. The pulmonary veins are very important: they carry the “red” blood that has picked up oxygen in the lungs back to the heart to be pumped out to the body. There are typically four pulmonary veins, two from each lung. Normally, they all connect directly into the heart’s left atrium (left upper chamber).

In TAPVR, there is a problem in the normal connection between the blood vessels coming from the lungs to the left atrium. This problem can take many different forms. In some cases, the blood vessels coming from the lungs connect with the right atrium instead of the left. Or the blood vessels from the lungs might drain into another blood vessel that subsequently drains into the right atrium instead of the left. In all cases, the blood that goes out to the body has less oxygen than normal. That can cause major symptoms and too much blood congestion in the lungs. In some forms of TAPVR, the blood vessels of the lungs can become squeezed (compressed) and blocked by nearby structures or because of the abnormal course they take. This can severe limit blood flow. If not immediately addressed, life-threatening consequences can occur.

Types of Total Anomalous Pulmonary Venous Return

There are typically 4 types of TAVPR:

  • Supracardiac: The pulmonary veins from both lungs typically drain to a chamber just behind the left atrium and then to additional veins that bring the blood to the right atrium.
  • Cardiac: The pulmonary veins from both lungs typically drain to a different part of the heart, rather than the left atrium.
  • Infracardiac: The pulmonary veins from both lungs typically drain into a common vein. Over time this connects to one of the major veins of the body. This vein is below the muscle that separates the chest and belly (diaphragm).
  • Mixed: The pulmonary veins from both lungs typically drain in a combination of the above types.

Causes of Total Anomalous Pulmonary Venous Return

Doctors know that heart defects present at birth (congenital) arise from errors early in the heart's development, but there's often no clear cause.

Symptoms of Total Anomalous Pulmonary Venous Return

Some people born with this condition have no signs or symptoms. However, as the condition worsens symptoms start to appear. Some of these symptoms may include:

  • A bluish discoloration of the lips and skin caused by low oxygen (cyanosis)
  • Rapid breathing or working harder while breathing, especially while eating
  • Unusual fatigue or lethargy

Diagnosis of Total Anomalous Pulmonary Venous Return

To diagnose this condition, your doctor will perform a variety of tests, including but not limited to:

Diagnostic tests and procedures

Treatment of Total Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return requires open heart surgery in all cases. Critically ill newborns will have surgery immediately. If the child is not critically ill, doctors may wait up to two months to perform surgery, depending on the strength of the child and the heart anatomy. Some treatment options include:

Medical and surgical procedures



Newark Beth Israel Medical Center
201 Lyons Avenue at Osborne Terrace
Newark, NJ 07112
(973) 926-7000
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Children's Hospital of New Jersey at Newark Beth Israel Medical Center
201 Lyons Avenue at Osborne Terrace
Newark, NJ 07112
(973) 926-7000
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