Total Anomalous Pulmonary Venous Return (TAPVR)

Nemours Cardiac Center at Nemours/Alfred I. duPont Hospital for Children

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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most.

At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).

If your child’s been diagnosed with total anomalous pulmonary venous return (or “TAPVR,”  also called “total anomalous pulmonary venous connection, or “TAPVC”), we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.

 
What Is It?

Total anomalous pulmonary venous return is a relatively rare abnormality in which the pulmonary veins that drain the lungs don’t connect to the heart in the usual way. Usually, the pulmonary veins bring oxygen-rich blood from the heart back to the left atrium where it can be pumped out to the body.

In total anomalous pulmonary venous return, the veins may connect in a variety of ways but, in all of them, the oxygen-rich blood from the lungs ends up in the right atrium. In order to get that blood into the left side of the heart where it can be pumped out to the body, there must be a hole (called an “atrial septal defect” or “ASD”) between the upper chambers of the heart.

Types of Total Anomalous Pulmonary Venous Return

The types of TAPVR are categorized based on the location of the drainage:

  • infracardiac TAPVR — when the pulmonary veins drain into veins (usually in the liver) that bring blood back from the lower body and abdomen
  • intracardiac TAPVR — when the pulmonary veins drain directly into the heart, but into the right atrium instead of the left, often through a connection to the vein which drains blood from the heart muscle itself (called the “coronary sinus”)
  • supracardiac TAPVR — when the pulmonary veins drain into the veins which bring oxygen-poor blood back from the upper body and head

The abnormal connection of the pulmonary veins may be wide open, in which case the main problem is that the oxygen-rich and oxygen-poor blood are mixing before being pumped out to the body. The level of oxygen in the body (called the “oxygen saturation”) is then low, so children may appear bluish (called “cyanosis”).

Sometimes the connection is too tight (or “obstructed”). In “obstructed total anomalous pulmonary venous return,” children still have low oxygen levels in the body, but the restriction to flow can also cause problems with normal development of the blood vessels in the lungs and can lead to high blood pressure in the lungs (called “pulmonary hypertension”).

How Does the Heart Normally Work?

When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »

Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:

 
How & When Is It Diagnosed?

Total anomalous pulmonary venous return may be diagnosed during a fetal echocardiogram, but it’s also commonly diagnosed immediately after birth. The exact timing depends on the severity of the obstruction (if present) and the anatomy of the abnormal connection. Newborns with severely obstructed veins will look extremely ill because blood can’t get through the pulmonary veins and back to the heart. These babies may have both low oxygen levels (“hypoxia”) and low blood pressure “hypotension,” with the heart unable to pump enough blood to the body.

At the Nemours Cardiac Center, we can make the initial diagnosis using an echocardiogram (“echo”). An echo is a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart. Children also may need a CT scan or MRI to completely identify the drainage of all portions of both lungs.

Learn More About Diagnostic Tests »

 
How & When Is It Treated?

To treat Total anomalous pulmonary venous return, Nemours Cardiac Center surgeons make a connection between the pulmonary veins and the left atrium, recreating the normal flow of blood (we close the atrial septal defect at the same time).

In children without any obstruction, surgical repair can safely be delayed, but all children will need an operation. Usually, our heart surgeons perform corrective surgery within the first six weeks after a baby’s birth.

Newborns who are very ill with severely obstructed pulmonary veins need an operation immediately, usually within four to six hours after birth. Occasionally, they may become so sick so fast that it’s better to use extra-corporeal membrane oxygenation (or “ECMO”) to help very sick infants recover so that they can tolerate a heart operation. (ECMO is a life-saving technique that replaces the function of the heart and lungs, allowing the child’s heart and lungs to rest and the organs to recuperate. During ECMO treatment, the heart continues to beat but a mechanical pump does most of the work.)

At the Nemours Cardiac Center, we offer everything from repair within a few hours of birth to the use of extracorporeal membrane oxygenation (or “ECMO”) devices.


Learn More
 
How Will It Affect My Child Long Term?

Most children do well following repair of total anomalous pulmonary venous return. In some cases, the area where the surgeon has created the new connection between the veins and the left atrium may become too tight (or “stenotic”), or the veins may not grow as the child grows. In these cases, children may need additional surgery or catheter procedures to treat the narrowed area. In all cases, children with total anomalous pulmonary venous return will need to be followed by a pediatric cardiologist to monitor the surgical repair and make sure children continue to do well.   

If your child has total anomalous pulmonary venous return, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More About Congenital Heart Defects »

Why Choose Us

From our outcomes to our family-centered care, find out all of the reasons why your child’s heart will be in good hands at the Nemours Cardiac Center. Learn More »

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Information for Patients

Outpatient Services and Inpatient Units: (302) 651-6660

After 5 p.m. and Weekends:

Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690


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Luke's Story

Meet Luke, who began showing signs of distress almost immediately after birth. He was rushed to the nursery, where tests showed that he was getting only 20 percent of the oxygen he needed. Read His Story »