Total Anomalous Pulmonary Venous Return

What is TAPVR?

Total anomalous pulmonary venous return (TAPVR) happens when veins (blood vessels) that bring blood from your baby’s lungs connect to the wrong place in their heart. As a result, their heart can’t put enough oxygen into the blood it sends to the rest of their body. TAPVR is a life-threatening heart condition. It’s congenital, which means it’s present at birth.

Some of the time, babies with total anomalous pulmonary venous return have trouble breathing soon after birth. Their skin may appear blue (or gray on darker skin).

All children with TAPVR also have an atrial septal defect (ASD). An ASD is a hole between your heart’s right and left atria.

Babies who have TAPVR need an operation. With early surgery, most children with TAPVR survive into adulthood. But some will need repeat surgery or procedures to treat narrowing in their veins later in life. Because of this, people with TAPVR need to see a cardiologist (a heart expert) regularly to monitor their health following surgery.

You may hear a provider call this condition total anomalous pulmonary venous connection (TAPVC).

TAPVR types

TAPVR types differ by how blood reaches the wrong place — your baby’s right atrium. Typically, your lungs send oxygen-rich blood to your heart’s left atrium (the chamber in your heart’s upper left side). In babies with TAPVR, the oxygen-rich blood flows through pulmonary veins to their heart’s right atrium instead.

In the right atrium, the oxygen-rich blood mixes with blood that doesn’t have as much oxygen. This low-oxygen blood travels out of the heart to the rest of your baby’s body.

The TAPVR types are:

• Supracardiac TAPVR: Blood flows via an ascending vertical vein into the brachiocephalic vein and then through their superior vena cava (a large vein in their body).
• Cardiac TAPVR: Blood moves through their coronary sinus. This vein normally drains blood that contains low levels of oxygen.
• Infracardiac TAPVR: The connecting vertical vein comes from their liver and inferior vena cava.
• Mixed TAPVR: A combination of veins from your baby’s lungs drain to more than one of the types mentioned above. This is the rarest form and the most difficult one to repair successfully.

How common is total anomalous pulmonary venous return?

Total anomalous pulmonary venous return is a rare condition. It affects 1 in about 7,500 newborns in the U.S. each year.

What are the symptoms of total anomalous pulmonary venous return?

Symptoms of TAPVR usually appear very soon after birth. Babies have symptoms at birth if they have any narrowing where their lung veins connect to their hearts. But some babies don’t have symptoms for several weeks.

Symptoms may include:

• Cyanosis (bluish or grayish skin, nails and lips).
• Difficulty feeding.
• Fatigue and lethargy (lack of energy).
• Heart murmur.
• Shortness of breath (dyspnea) and trouble breathing.
• Lack of growth or weight gain.

What causes total anomalous pulmonary venous return?

Healthcare providers aren’t sure what causes total anomalous pulmonary venous return. It happens when your baby’s heart and blood vessels are forming in the uterus during fetal development.

This doesn’t seem to be an inherited condition (passed down through families). But there have been similar cases among siblings. TAPVR has an association with certain syndromes and exposure to paint removers, pesticides and lead.

What are the complications of total anomalous pulmonary venous return?

A baby with TAPVR can have heart failure and pulmonary hypertension. Even after the initial repair, TAPVR can sometimes lead to abnormal heart rhythms or blockages in your child’s pulmonary veins.

How is total anomalous pulmonary venous return diagnosed?

Cardiologists can sometimes diagnose this condition with an echocardiogram before birth. Some babies don’t get a diagnosis until they’re several weeks or months old.

After your baby is born, their provider will do a physical exam and listen to your baby’s heart. A pulse oximeter (pulse ox) can measure the amount of oxygen in your baby’s blood. It fits over your baby’s big toe and sends information through a wire.

To see pictures of your child’s heart, a provider may use:

• Chest X-ray.
• CT (computed tomography) scan.
• Heart MRI (magnetic resonance imaging).
• Echocardiogram.
• Electrocardiogram (EKG or ECG).

Some of these tests (MRI) may require anesthesia, but they’re all noninvasive. They allow your baby’s healthcare provider to see images of your baby’s heart and veins. They also help them evaluate blood flow and look for abnormalities.

A provider can use cardiac catheterization to make a diagnosis. But they can usually get the information they need from imaging. This spares your baby from an invasive procedure.

How is total anomalous pulmonary venous return treated?

Nearly every baby with total anomalous pulmonary venous return needs surgery to survive. Healthcare providers treat TAPVR with open-heart surgery. Most often, providers perform this surgery as soon as they can after diagnosing the condition.

Though it’s extremely rare, some people don’t have surgery as babies and get a TAPVR diagnosis as adults. But they usually have high pressures within their lungs (pulmonary hypertension) that can make surgical repair very challenging.

While waiting for surgery, your child may receive extra oxygen or a ventilator to help them breathe. They may receive an inotrope, which is a medicine that helps their heart beat more forcefully.

While your baby is asleep under general anesthesia, a surgeon:

1. Makes cuts (incisions) in your baby’s chest and heart.
2. Connects the pulmonary veins to the correct place (the left atrium) in your baby’s heart.
3. Closes abnormal connections.
4. Removes any blockage in your child’s pulmonary vein.
5. Most often, closes the hole between their left and right atria (atrial septal defect).

Complications of the treatment

Possible complications after surgery include:

• Arrhythmias.
• Pulmonary hypertension.
• Pulmonary venous obstruction.
• Inability of the heart to beat on its own after the use of a bypass machine.

Can total anomalous pulmonary venous return be prevented?

No. It isn’t possible to prevent this congenital heart condition. If you have a family history of congenital heart conditions or you have TAPVR, talk to your healthcare provider before starting a family.

What can I expect if my child has total anomalous pulmonary venous return?

Without surgery, some forms of total anomalous pulmonary venous return are typically fatal a few weeks after birth. With early diagnosis and surgical treatment, the outlook for babies with TAPVR is very good. The survival rate after surgery is around 97%.

Your child will need regular visits with their cardiologist as they grow into adulthood. Lifelong follow-up visits can help cardiologists find problems like an irregular heartbeat or blockages (obstructions) in their blood vessels. An obstruction requires another surgery and may be hard to treat.

Your child may need to take medicine or have a procedure like a cardiac catheterization.

How do I take care of my child?

Children with TAPVR may have trouble with typical development like fine motor function. You can help your child by getting screenings for developmental milestones. If a screening shows a developmental issue, you can arrange for someone to work with them to improve their skills.

Talk to your child’s healthcare provider about their ability to participate in sports and physical activities. Your child may need to limit vigorous exercise.

When should my child see their healthcare provider?

After surgery, your baby will have checkups every six to 12 months. They’ll need regular follow-up appointments through adulthood. Your child’s provider may want to order tests like an electrocardiogram, exercise stress test or echocardiogram.

When should I go to the ER?

Get medical help right away if your baby:

• Has bluish or grayish skin, nails or lips.
• Has trouble breathing or difficulty eating.
• Seems lethargic.

What questions should I ask my doctor?

Questions to consider asking your child’s healthcare provider may include:

• What type of TAPVR does my child have?
• Do you think my child will need a second operation?
• How many times have you performed surgery to repair TAPVR?

A note from Cleveland Clinic

You’ve carefully followed your provider’s instructions for a healthy pregnancy. But somehow, your newborn has a heart issue. Today’s medical advances give your baby a better outlook than for previous generations. Surgical treatments are extremely effective. The outlook for people with this condition is very good. Following surgery, be sure to take your child to see their healthcare provider for regular follow-up visits. These visits help your child’s provider treat problems and monitor their health.