What is total anomalous pulmonary venous return (TAPVR)?

Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect. This means that your child is born with it. It happens as the baby’s heart develops during the first 8 weeks of pregnancy.

In this condition, the 4 blood vessels (pulmonary veins) that carry oxygen-rich (red) blood to the heart from the lungs aren’t connected correctly. Normally these blood vessels should be connected to left upper chamber (atrium) of the heart. Instead, they’re connected somewhere else.

There are different types of TAPVR. The type your child has depends on where the pulmonary veins are connected.

This condition causes oxygen-rich blood from the lungs to mix with oxygen-poor (blue) blood from the body. This keeps oxygen-rich blood from being carried all over the body. Your child can't live with TAPVR long-term. They need oxygen-rich blood all over the body.

Other heart defects often occur with TAPVR. They actually help infants have enough oxygen in their blood until they can have surgery. These other defects include:

• Atrial septal defect. In this condition, an opening is between the 2 upper chambers of the heart (atrial septum). This causes oxygen-poor and oxygen-rich blood to be mixed.

• Patent ductus arteriosus (PDA).  The ductus arteriosus is a blood vessel that carries blood away from the heart in a developing baby. It often closes soon after birth. If it stays open, it’s called a PDA. Sometimes it stays open with TAPVR. This causes oxygen-poor and oxygen-rich blood to be mixed.

• Single ventricle. In this condition, there is only 1 ventricle instead of the normal 2.

What causes TAPVR?

Most of the time, the cause of TAPVR isn’t known. It may occur with other heart or body system problems.

What are the symptoms of TAPVR?

The symptoms of TAPVR may seem like symptoms of other health conditions. Your child’s healthcare provider must diagnose them with this condition.

Newborns with TAPVR will have blue coloring of their skin, lips, and nailbeds (cyanosis). This happens in the first hours or days of their life. How severe your child’s condition is depends on how much blood is able to get to their body.

Other symptoms can be a bit different for each child. They can include:

• Fast or troubled breathing

• Fast heart rate

• Cool, clammy skin

• Tiredness and little movement

• Poor feeding

• Poor pulses

• Enlarged liver

• Heart murmur

How is TAPVR diagnosed?

A healthcare provider may first spot TAPVR in a baby during an ultrasound in pregnancy. After birth, if your child has signs of TAPVR, a pediatric cardiologist or neonatologist will check them. A pediatric cardiologist is a doctor with special training to diagnose and treat heart problems in babies and children. A neonatologist is a doctor with special training to diagnose and treat problems in newborns. These include babies born premature and full-term.

Next, your child’s healthcare provider may do tests to check for heart problems. The tests your child needs depend on their age and condition. They also depend on the provider’s preferences.

Chest X-ray

A chest X-ray may show changes in the heart and lungs caused by TAPVR.

Electrocardiogram (ECG)

An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and finds heart muscle stress. These problems may be caused by TAPVR.

Echocardiogram (echo)

This test uses sound waves to make a moving picture of the heart and heart valves. An echo can show the structural changes of TAPVR and abnormal connections of the pulmonary veins.

Cardiac catheterization

A cardiac catheterization gives detailed information about the structures in the heart. In this test, a small, thin, flexible tube (catheter) is put into a blood vessel in your child’s groin. Then the healthcare provider guides it to your child’s heart. There, they will inject your child with contrast dye to see the heart more clearly. Your child’s provider will give them medicine to help relax and prevent pain (sedation). Your child’s blood pressure and oxygen levels will be checked during the procedure. Your child may not need this test if a diagnosis is made during the echo.

CT and magnetic resonance angiography (MRA)

A CT and MRA can show detailed information about the pathways and connections of the pulmonary veins.

How is TAPVR treated?

Treatment will depend on your child’s symptoms, age, and overall health. It will also depend on how severe the condition is.

All children with a TAPVR will need to have surgery to fix it. Your baby may need to go into the intensive care unit (ICU) for treatment.

Medical management

At first, your baby may get the following care:

• Supplemental oxygen or a machine that helps your baby breathe (ventilator).

• Prostaglandin therapy. This medicine keeps the ductus arteriosus open and lets blood flow through the heart. This medicine isn’t used in all types of TAPVR.

• Different medicine to support the function of the heart.

• ECMO (extracorporeal membrane oxygenation). This method gives both cardiac and respiratory support for babies who can't maintain oxygen levels on a ventilator.

Cardiac catheterization

This procedure can be used to diagnose and treat some heart defects. To treat TAPVR, your child’s healthcare provider may do a test called a balloon atrial septostomy. This test makes it easier for oxygen-rich blood to get to the left side of the heart and then the rest of the body.

Surgery

The surgery is done through a cut (incision) through the breastbone (sternum) and into the chest. Your baby will be connected to a heart-lung machine during surgery. This device does the work of your baby's heart and lungs during surgery. The details of the surgery depend on the type of TAPVR your baby has. Your child’s healthcare provider will explain the procedure to you. Some babies with severe forms of TAPVR may need surgery shortly after birth.

What are possible complications of TAPVR?

The complications of TAPVR include:

• Enlarged heart

• Lung problems, including respiratory failure and high blood pressure in the lungs

• Abnormal heart rhythms

• Shock

• Heart failure

• Slowed growth and development

• Enlarged liver

• Need for another surgery

• Infection

• Death

Living with TAPVR

Your child will likely stay in the hospital until TAPVR is repaired. Your child’s healthcare team will give you directions about special treatments or medicines your child needs before your child leaves the hospital. For instance, your child may need special formula and supplemental feedings to help with their growth. Your child’s healthcare team will also help you plan for home healthcare if you need it.

Many children with TAPVR will grow and develop normally after they have surgery. Talk with your child's cardiologist about how much and what kinds of physical activity your child can safely do. Your child will still need to see a heart doctor for regular checkups for the rest of their life. Your child’s follow-up care should be at a center that specializes in congenital heart defects. Ask your child's healthcare provider about your child’s outlook.

Your child may also need the following care:

• As children grow, they may need more surgeries and cardiac catheterizations.

• Your child's heart doctor may give your child antibiotics before surgeries or dental procedures. This is to prevent infections. But this often isn’t needed.

• Some children have delayed development. Talk with your child’s healthcare provider about their development. Your child may need to be checked for issues.

When should I call my child's healthcare provider?

Call your child's healthcare provider if:

• Your child's skin, lips, or fingernails become blue

• Your child has trouble breathing or feeding

Key points about TAPVR

• In TAPVR, the 4 blood vessels that carry oxygen-rich blood to the heart from the lungs aren’t connected correctly.

• This condition prevents oxygen-rich blood from being pumped to the body.

• Your child can't live with TAPVR long-term because people need oxygen-rich blood all over their body.

• All children with TAPVR will need surgery. This is done to restore normal blood flow through the heart.

• Many children with TAPVR will grow and develop normally after they have surgery. 

• Your child will need to see a heart doctor for regular checkups for the rest of their life.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.

• Before your visit, write down questions you want answered.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new directions your provider gives you for your child.

• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.

• Ask if your child’s condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if your child does not take the medicine or have the test or procedure.

• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your child’s healthcare provider after office hours. This is important if your child becomes ill and you have questions or need advice.