Total anomalous pulmonary venous return

Total anomalous pulmonary venous return (TAPVR) is a heart disease in which the 4 veins that take blood from the lungs to the heart do not attach normally to the left atrium (left upper chamber of the heart). Instead, they attach to another blood vessel or the wrong part of the heart. It is present at birth (congenital heart disease).

Congenital heart disease

Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth.

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Causes

The cause of total anomalous pulmonary venous return 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 (lung) veins to the left side of the heart, which sends blood out through the aorta and around the body.

In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, 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.

For the infant to live, an 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 the rest of the body.

Atrial septal defect

Atrial septal defect (ASD) is a heart defect that is present at birth (congenital). As a baby develops in the womb, a wall (septum) forms that divide...

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Patent foramen ovale

Patent foramen ovale (PFO) is a hole between the left and right atria (upper chambers) of the heart. This hole exists in everyone before birth, but ...

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How severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery.

Symptoms

The infant may appear very sick and may have the following symptoms:

• Bluish color to the skin (cyanosis) due to low oxygen level in the blood

  Bluish color to the skin (cyanosis)

  A bluish color to the skin or mucous membrane is usually due to a lack of oxygen in the blood. The medical term is cyanosis.

  

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• Frequent respiratory infections
• Lethargy
• Poor feeding
• Poor growth
• Rapid breathing

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

Exams and Tests

Tests may include:

• Cardiac catheterization can confirm the diagnosis by showing that the blood vessels are abnormally attached

  Cardiac catheterization

  Cardiac catheterization involves passing a thin flexible tube (catheter) into the right or left side of the heart. The catheter is most often insert...

  

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• ECG shows enlargement of the ventricles (ventricular hypertrophy)

  ECG

  An electrocardiogram (ECG) is a test that records the electrical activity of the heart.

  

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• Echocardiogram may show that the pulmonary vessels are attached

  Echocardiogram

  An echocardiogram is a test that uses sound waves to create pictures of the heart. The picture and information it produces is more detailed than a s...

  

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• MRI or CT scan of the heart can show the connections between the pulmonary vessels

  MRI

  Heart magnetic resonance imaging is an imaging method that uses powerful magnets and radio waves to create pictures of the heart. It does not use ra...

  

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  CT scan

  A computed tomography (CT) scan of the heart is an imaging method that uses x-rays to create detailed pictures of the heart and its blood vessels. Th...

  

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• X-ray of the chest shows a normal to small heart with fluid in the lungs

  X-ray of the chest

  A chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.

  

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Treatment

Surgery to repair the problem is needed as soon as possible. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.

Outlook (Prognosis)

If this condition is not treated, the heart will get larger, leading to heart failure. Repairing the defect early provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart. Infants who have obstructed veins have worsened survival.

Possible Complications

Complications may include:

• Breathing difficulties
• Heart failure

  Heart failure

  Heart failure is a condition in which the heart is no longer able to pump oxygen-rich blood to the rest of the body efficiently. This causes symptom...

  

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• Irregular, fast heart rhythms (arrhythmias)
• Lung infections
• Pulmonary hypertension

When to Contact a Medical Professional

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

Contact your health care provider if you notice symptoms of TAPVR. Prompt attention is required.

Prevention

Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease.

Women who are pregnant should get good prenatal care:

• Avoid alcohol and illegal drugs during pregnancy.
• Tell your provider that you are pregnant before taking any new medicines.
• Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery.
• Pregnant women who have diabetes should try to get good control over their blood sugar level.

Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.