Partial Anomalous Pulmonary Venous Return

What is partial anomalous pulmonary venous return?

Partial anomalous pulmonary venous return (PAPVR) is a rare form of congenital heart disease (congenital means you’re born with it). It affects the flow of oxygen-rich blood from your lungs to your heart. Your pulmonary veins normally return all freshly oxygenated blood to the left side of your heart. But in PAPVR, they deliver some of this blood to the right side, which should only hold oxygen-poor blood.

This leads to a higher volume of blood than normal in your right heart chambers, which have to work harder to compensate. This extra work may take a toll on your heart and your overall health.

PAPVR affects people differently depending on how much blood goes to the wrong place. You may have a mild form of the condition and never develop symptoms. But if you do develop symptoms or complications, healthcare providers can manage your condition. Surgery is typically successful at treating PAPVR and helping you live a long and healthy life.

Normal blood flow vs. PAPVR blood flow

To understand how PAPVR affects blood flow, it helps to learn how blood normally flows through your heart and lungs. Here’s a breakdown:

1. Oxygen-poor blood from your body flows into two large veins called your superior and inferior vena cava. These veins empty directly into your heart’s right atrium (top right chamber).
2. The oxygen-poor blood flows from your right atrium into your right ventricle.
3. Your right ventricle pumps the blood to your lungs through your pulmonary arteries. In your lungs, the blood gains oxygen and is ready to go back to your heart.
4. The oxygen-rich blood travels through several pulmonary veins to return to your heart, entering at your left atrium.
5. The oxygen-rich blood flows into your left ventricle.
6. Your left ventricle pumps this blood out to your body through your aorta.

Notice that normally, oxygen-poor blood stays in the right chambers, and oxygen-rich blood stays in the left chambers.

With PAPVR, this system doesn’t work as it should. PAPVR creates a left-to-right cardiac shunt, or an atypical pattern of blood flow through your heart. “Partial anomalous” means some (but not all) of the oxygen-rich blood from your lungs doesn’t return to your heart in the normal way.

This is because one or more of your pulmonary veins carry some oxygen-rich blood into your right atrium, rather than your left atrium. So, your right atrium contains a mix of oxygen-poor and oxygen-rich blood. Your right ventricle has no choice but to pump this mixed blood out to your lungs.

This means two things are happening that shouldn’t. First, some oxygen-rich blood unnecessarily returns to your lungs, where it just came from. Second, your right ventricle has a higher load of blood it must pump to your lungs. This extra work can take a toll on your heart over time.

PAPVR with other heart disorders

Some people who have PAPVR have no other heart disorders. This is called isolated PAPVR. However, many people with PAPVR also have other anomalies such as an atrial septal defect (ASD).

Rarely, PAPVR occurs along with an underdeveloped right lung and pulmonary artery (scimitar syndrome).

How rare is partial anomalous pulmonary venous return?

Researchers estimate that about 4 to 7 in 1,000 people have PAPVR. This number may be higher, though, since some people never show symptoms and therefore may never receive a diagnosis.

What are the symptoms of PAPVR?

PAPVR doesn’t always cause symptoms when the volume of abnormal blood flow (shunting) is low. But you may notice symptoms as the shunting increases. Children may have symptoms if they have other heart conditions, but many times, people with PAPVR don’t have symptoms until their 20s or 30s.

Symptoms in children can include:

• Delays in growth.
• Easily becoming tired during exercise.
• Recurrent respiratory infections.
• Shortness of breath.
• Trouble gaining weight.

PAPVR symptoms in adults can include:

• Shortness of breath.
• Fatigue.
• Heart palpitations.
• Chest pain.
• Rapid heart rate.
• Recurrent respiratory infections.

What causes PAPVR?

PAPVR, like other forms of congenital heart disease, happens when a fetal heart doesn’t form properly in the uterus.

Normally, during the early weeks of pregnancy, four pulmonary veins form out of a common pulmonary vein. These veins connect with the fetal left atrium. However, changes in this process cause the pulmonary veins to connect to the right atrium or nearby veins (like the superior vena cava or left brachiocephalic vein).

What are the complications of PAPVR?

Over time, uncorrected left-to-right shunts can reverse and cause Eisenmenger syndrome.

How is PAPVR diagnosed?

Healthcare providers diagnose PAPVR through a physical exam and testing. During an exam, your provider uses a stethoscope to listen to your heart. Some people with PAPVR have a heart murmur. If your provider suspects a problem, they’ll order further testing.

The age of diagnosis for PAPVR varies widely from infancy through late adulthood. You can live with PAPVR for much of your life but not know it because you have no symptoms. Sometimes, testing for another purpose reveals the anomaly.

What tests diagnose this condition?

Tests that help providers diagnose PAPVR include:

• Echocardiogram, especially a transesophageal echocardiogram (TEE).
• Heart MRI.
• Cardiac computed tomography (CT) scan.

Does PAPVR require surgery?

Some children and adults need PAPVR repair surgery to reroute blood flow through their heart. Children diagnosed with PAPVR are more likely than adults to need surgery. This is because they often have additional heart disorders that require surgical treatment.

Providers only recommend surgery if it’s necessary to ease symptoms or prevent complications. You or your child may need surgery if PAPVR is causing significant left-to-right shunting. This means a considerable amount of blood that should go out to your body is recirculating through your right heart and lungs. This can cause uncomfortable symptoms or lead to complications (like PAH or heart failure). Your provider will use imaging tests to determine the extent of shunting.

Surgery can typically treat PAPVR successfully. It’s important to speak with your provider about the details of your surgery and what it will accomplish. The techniques can vary depending on the anatomy of your heart and pulmonary veins. In general, surgeons aim to reroute blood flow so that oxygen-rich blood ends up in your left heart chambers.

Many people don’t need surgery. If your PAPVR is mild, your provider will keep an eye on the situation. If you develop symptoms or signs of complications, your provider may recommend surgery at that point.

Can PAPVR be prevented?

Unfortunately, you can’t prevent PAPVR. It’s something you’re born with. However, if you’re planning a pregnancy, talk to your provider about ways to lower your risk of delivering a baby with congenital heart disease. Some tips include:

• Avoid beverages containing alcohol, recreational drugs, tobacco products and secondhand smoke.
• Keep all of your prenatal appointments with your provider.
• Work with your provider to manage underlying health conditions.

What can I expect if I have this condition?

It’s possible to live a long and healthy life with PAPVR. Many people live for many years without any symptoms or complications. Those who need surgery typically have good outcomes. However, outcomes can vary depending on other heart issues or underlying conditions that a person may have. So, you should talk to your provider about what to expect in your individual situation.

How do I take care of myself?

Living with congenital heart disease can be stressful. It’s important to remember to take one day at a time. Here are some general tips you can follow:

• Establish care with a cardiologist who specializes in adult congenital heart disease. They’ll monitor your condition through regular check-ups and testing.
• Ask your provider what kinds of exercise are suitable for you. Together, create an exercise plan.
• Follow a heart-healthy diet like the Mediterranean Diet.
• Take any medications as your provider prescribes them.
• Avoid smoking and all tobacco products. Ask your provider for resources to help you quit.

If your child has PAPVR, ask their provider how best to care for them. Your child may need to follow special guidelines for exercise or other activities, particularly if they also have other forms of congenital heart disease.

When should I see my healthcare provider?

Visit your provider according to the schedule they recommend. Routine follow-ups and imaging tests are important for keeping an eye on PAPVR and managing complications.

Call your provider if you or your child:

• Have new or changing symptoms.
• Have side effects from medications.
• Have any questions or concerns.

When should I go to the ER?

Call 911 or your local emergency number if you or your child experiences:

• A fast heart rate (100-150 beats per minute) that won’t go down.
• Fainting spells with loss of consciousness.
• Shortness of breath that’s out of proportion to exercise and/or doesn’t go away when you rest.
• Sudden and severe chest pain.
• Sudden and severe headache.
• Sudden weakness or paralysis in your arms or legs.

What questions should I ask my doctor?

Talk to your provider about your condition and what you can expect going forward. Some questions you may want to ask include:

• How serious is my condition?
• What lifestyle changes should I make?
• Will I need surgery?
• What are the benefits and the risks of surgery?
• What is my outlook?

If your child has PAPVR, ask their provider:

• How serious is my child’s condition?
• Does my child have other congenital heart disorders?
• Will my child need surgery or other treatment?
• How can I take care of my child at home?
• Do they have any activity restrictions?
• What age-appropriate resources can you share with my child?

What is the difference between PAPVR and TAPVR?

PAPVR is different from total anomalous pulmonary venous return (TAPVR). With TAPVR, all of your pulmonary veins connect abnormally to your heart. TAPVR is more serious than most forms of PAPVR and requires more urgent treatment, usually soon after birth. However, PAPVR and TAPVR are similar in that both disrupt normal blood flow through your heart.

A note from Cleveland Clinic

PAPVR may not affect your life at all. Or, it may cause symptoms or complications that require treatment. No matter how it impacts your day-to-day routine, PAPVR might make you feel stressed or anxious. If your child has PAPVR, you may worry about their well-being. These feelings are common and normal.

Share your thoughts with your healthcare provider. Ask what resources are available to you and your family. Learning more about congenital heart disease — whether it affects you, your child or another loved one — can help you better manage the situation. Speaking with a counselor may also help you work through your thoughts. Along the way, don’t hesitate to reach out to your provider with any questions or concerns that come to mind.