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Total Anomalous Pulmonary Venous Return

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Having a child with a heart condition such as total anomalous pulmonary venous return (TAPVR) can be worrisome. When you come to Children’s Health℠ you can be confident they’ll get the best care possible. Physicians at Children’s Health in conjunction with world-class subspecialists from UT Southwestern provide expert care for children with this rare condition.

TAPVR occurs when your child’s pulmonary vein connects to the top right of their heart (atrium) instead of the left. Instead of keeping blood flows separated, TAPVR allows oxygen-rich blood to mix with oxygen poor blood. This “mixed” blood does not have enough oxygen in it to support your child’s needs, creating a life-threatening condition.

Children with TAPVR often have other heart defects including atrial septal defect, in which a hole between the left- and right atrium allows mixed blood to pass to the left side of the heart where it is pushed out to the body. The good news is that surgery can treat both defects.

Symptoms

Symptoms of Total Anomalous Pulmonary Venous Return

In some cases, you may know your child has TAPVR before he or she is born. Some symptoms may show up during routine prenatal tests, such as a fetal ultrasound. However, most children develop symptoms within their first year of life.

These symptoms include:

  • Skin that appears ashen or blue from poor circulation (cyanosis)
  • Rapid breathing or shortness of breath
  • Heart murmur
  • Weak pulse
  • Difficulty feeding
  • Pounding heart
  • Sleeping more than usual

These symptoms may start showing at birth, especially when a child’s pulmonary veins are narrow or blocked. If your child’s veins are not narrowed, he or she may not start showing signs for a few weeks or even months.

Tests and Diagnosis

Diagnosing Total Anomalous Pulmonary Venous Return

While it is possible to diagnose TAPVR during pregnancy, it’s more common to diagnose these defects after a baby is born. Accurately diagnosing TAPVR helps us make sure your child gets the treatment he or she needs as soon as possible – even on their first day of life.

Your child’s physical evaluation may include:

  • Questions about growth, development, family history and any recent illnesses
  • Listening to the heart and lungs
  • Observing symptoms

Diagnostic testing may include:

  • Electrocardiogram
  • Echocardiogram
  • Cardiac MRI
  • Chest X-ray
  • Cardiac catheterization
  • Pulse oximetry screening

At Children’s Health℠, we perform diagnostic tests using special imaging equipment designed with our smallest patients in mind. Learn more about the full spectrum of tests available from our cardiac imaging department.

Treatments

Treating TAPVR

Children with TAPVR need surgery to repair their defects and restore normal blood flow through the heart.

Corrective surgery includes:

  • Connecting the pulmonary veins to the left atrium
  • Closing off any abnormal connections between blood vessels
  • Closing your child’s atrial septal defect

Exactly when your child has surgery depends on how serious their condition is:

  • If your child is very sick (critically ill) he or she will have surgery right away
  • Children who are not critically ill may wait two to six months for surgery, depending on the structure of the abnormal connections as well as other symptoms

Your doctor will let you know when it’s best for your child to have surgery.

After surgery your child should be free of symptoms, however they are not cured of the disease. You can expect your child to live a healthy, active life but he or she will need regular follow up visits to check for complications and other heart conditions.

FAQs

Total Anomalous Pulmonary Venous Return FAQs

Here are answers to some frequently asked questions:

What causes TAPVR?

The cause of total anomalous pulmonary venous return is unknown.

My child was born healthy and now is very sick. What happened?

Children who have TAPVR without blocked vessels typically appear healthy at birth. Over time, the low levels of oxygen in their blood lead to symptoms that continue to get worse until your child has corrective surgery.

Does my child have to have surgery? Can we try other treatments first?

Unfortunately, the only effective treatment for TAPVR is corrective surgery. The good news is that after your child recovers he or she can expect to live a normal healthy life.

Will my child need more than one surgery?

It’s possible. After surgery, scar tissue may form around the connection between the pulmonary veins and the heart. If the scar tissue interferes with normal heart functioning, your child will need an additional procedure to remove it.

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