Total anomalous pulmonary venous drainage

What is total anomalous pulmonary venous return (TAPVR)?

Total anomalous pulmonary venous return (TAPVR) is a birth defect of the heart in which the four major blood vessels of the heart are improperly connected to the atria, or pumping chambers.

A healthy heart is a strong, muscular pump that continuously pushes blood through the circulatory system to deliver oxygen and nutrients to the body. A system of blood vessels carry blood to and from the four chambers of the heart to be oxygenated. Oxygen-poor (blue) blood returns from the body and is replenished by the lungs to become oxygen-rich (red) again.

In a heart with TAPVR, the pulmonary veins—the four major blood vessels that return to the heart from the lungs—are connected abnormally. The abnormal connections may appear in different places, but in most cases, the result is that oxygen-rich (red) blood that should go out to the body is instead returned to mix with oxygen-poor (blue) blood, and the body does not receive the oxygen it needs to function. The abnormal connection can be above, into or below the heart and can be either obstructed (narrowed) or unobstructed.

Often, children born with TAPVR have other heart defects that actually help the heart to function until treatment is possible. These other defects may include an atrial septal defect, a ventricular septal defect, a patent ductus arteriosus or very complex forms of heart disease called heterotaxy. Each of these defects is a type of hole in the heart that allows red and blue blood to mix within the heart so your baby's body may receive needed oxygen.

What causes TAPVR?

TAPVR occurs during the first eight weeks of fetal development when the heart is forming. Most of the time, this heart defect occurs by chance with no clear cause. Some congenital heart defects may have a genetic link, causing heart problems to occur more often in certain families. In some cases, environmental factors may also play a part. TAPVR occurs in about one out of every 15,000 live births, and occurs equally in boys and girls. In most cases, TAPVR cannot be prevented but it can be treated.

What are the signs and symptoms of TAPVR?

The symptoms of TAPVR vary for each child, but may commonly include:

  • Cyanosis (a persistent blue or gray tone to the skin, lips, or nails)
  • Pale, cool or clammy skin
  • Difficult or rapid breathing
  • Rapid heart rate
  • Poor appetite and insufficient weight gain
  • Unusual tiredness or irritability

After your baby is born, your doctor may suspect a heart defect if he or she hears a heart murmur — an abnormal whooshing noise, heard through a stethoscope exam, which may indicate a problem with blood flow. Most heart murmurs are called "innocent heart murmurs." Children with innocent heart murmurs do not have a heart defect and do not experience heart problems. However, if a heart murmur is present along with other symptoms, your doctor may want to investigate further by ordering other tests.

Depending on your child's specific condition, symptoms may appear shortly after birth or may take a few days or weeks to emerge.

How is TAPVR treated?

With prompt diagnosis and proper treatment, most children born with TAPVR can grow and develop normally.

Your child's specific treatment may vary, depending on his or her individual needs. However, surgical repair of TAPVR is typically necessary in early infancy. Before the surgery, your child will be admitted to the intensive care unit (ICU) and given intravenous medications to help heart and lung function. A ventilator may be used to assist breathing.

A clear diagnosis is the first step to treatment. A pediatric cardiologist (a children's heart doctor) can use several tests to confirm your child's diagnosis. These tests may include:

  • Chest X-ray: A beam of electromagnetic energy creates images on film that show the inside structures of your baby's body. A chest X-ray doesn't provide a definitive diagnosis, but it helps the doctor determine your baby's heart size and structure.

  • Electrocardiogram (ECG or EKG): This test, conducted by attaching patches with wires (electrodes) to the baby's skin, records the heart's electrical activity. It will show if there are abnormal heart rhythms (arrhythmias or dysrhythmias) and heart muscle stress.

  • Echocardiogram (Echo): This test uses high-pitched sound waves to produce a moving image of the heart on a video screen. It is similar to an ultrasound, and can be used to assess the structure and position of the parts of the heart, as well as heart defects. Echocardiogram is usually the definitive procedure for diagnosing TAPVR.

  • Cardiac catheterization: During this procedure, your doctor inserts a thin flexible tube (a catheter) into a blood vessel in the groin, then guides it up to the inside of the heart. A dye injected through the catheter makes the heart structures visible on x-ray pictures. The catheter also measures blood pressure and oxygen levels and can evaluate how much of the blood in the heart is mixing.

The cardiac catheterization process may be used to perform a balloon atrial septostomy by inflating a tiny balloon inside the left atrium of the heart and enlarging the defect or hole there to allow more blood to mix between the atria. Some time after this, surgery will be required to redirect the pulmonary veins to their proper connections.

Children who undergo surgery for TAPVR in early infancy typically grow and develop normally. However, your child will need lifelong follow-up care with a cardiologist (a heart doctor) to ensure that further obstructions do not develop, and to monitor and treat any heart rhythm irregularities.

About treatment for total anomalous pulmonary venous return at Children's

Total anomalous pulmonary venous return is treated through Children's cardiovascular program one of the largest and oldest pediatric cardiovascular programs in the region. Team members consistently achieve treatment results that are among the best in the nation. Each year, care is provided for thousands of the region¹s sickest children with heart conditions, including fetuses, newborns, infants, children, adolescents, and adult, long-term patients with pediatric cardiovascular conditions.

Contact Us

For more information, please call Children's Heart Clinic at 1-800-938-0301.

Some content for this fact sheet used by permission from It's My Heart, a publication of The Children's Heart Foundation, © 2006.