Anomalous pulmonary venous return 

Anomalous pulmonary venous return is a serious condition where lung veins connect to the heart in abnormal places. It’s usually diagnosed shortly after birth. Anomalous pulmonary venous return is also known as anomalous pulmonary venous connection.

This congenital heart defect usually requires surgery to treat. The ways in which the lung veins connect abnormally to the heart are different for each child, which makes each surgery different. At Advocate Children’s Hospital, you’ll have access to a team of world-class cardiologists and cardiovascular surgeons who have extensive experience in managing such complex surgeries.

What is anomalous pulmonary venous return?

To understand anomalous pulmonary venous return, it helps to start with what pulmonary veins are supposed to do.

Normally, there are four pulmonary veins that carry oxygen-rich blood from the lungs to the top chamber (atrium) on the left side of the heart. There are two left pulmonary veins that come from the left lung and two right pulmonary veins that come from the right lung.

After the oxygenated blood arrives, the heart pumps it to various parts of your baby’s body that need it. After the oxygen from the blood is delivered and used, the oxygen-poor blood is pumped back to the lungs, where it picks up oxygen for another trip through the heart and body.

Pulmonary veins are considered anomalous when, due to a birth defect, one or more of your baby’s pulmonary veins are connected to the heart at abnormal places. This decreases the amount of oxygen-rich blood that is pumped to the body. The anomalous veins can also become blocked and prevent blood from returning to the heart from the lungs.

What causes anomalous pulmonary venous return?

We don’t know what causes anomalous pulmonary venous return. It’s thought to occur early in pregnancy when the heart is being formed. It doesn’t seem to be an inherited condition, however there are sometimes similar conditions among siblings.

Anomalous pulmonary venous return sometimes occurs on its own. Or it can occur along with other congenital heart defects.

Total anomalous pulmonary venous return

When all your baby’s pulmonary veins are affected, it creates a rare condition called total anomalous pulmonary venous return (TAPVR).

Babies who have TAPVR always have an atrial septal defect, which means there’s a hole between the left atrium and right atrium of their heart. The hole allows some oxygenated blood to reach the left atrium and be pumped to the rest of their body. However, the blood that reaches the left atrium is a mixture of oxygen-rich blood and oxygen-poor blood. This means your baby’s body has to use blood that doesn’t have enough oxygen in it.

Some types of total anomalous pulmonary venous return can be life-threatening and have to be surgically repaired soon after birth.

What are the different types of TAPVR?

When your baby has total anomalous pulmonary venous return, it’ll be described as one of these types:

• Supracardiac: Pulmonary veins that connect to the heart abnormally after travelling above the heart. This is the most common anomaly (difference).
• Cardiac: Pulmonary veins that connect directly to the wrong part of the heart.
• Infracardiac: Pulmonary veins that travel below the diaphragm before connecting abnormally to the heart.
• Mixed: A mix of pulmonary vein connections that combine some or all the other types.

Total anomalous pulmonary venous return symptoms

Babies with TAPVR usually have symptoms shortly after birth, particularly if their pulmonary veins are narrowed where they connect to the heart. Sometimes total anomalous pulmonary venous return symptoms don’t appear for weeks. Symptoms of TAPVR in infants may include:

• Blue or gray skin, lips or nails
• Shortness of breath and trouble breathing
• Poor weight gain or growth
• Trouble feeding
• Fatigue or lack of energy
• Heart murmur

Partial anomalous pulmonary venous return

When only some of your baby’s pulmonary veins are affected, it’s called partial anomalous pulmonary venous return (PAPVR). They may not have symptoms at birth if the volume of blood that goes to the wrong place in the heart is low. Symptoms may start after months or years. Or they may not have symptoms until they become adults – starting in their 20s or 30s.

Some children or adults with PAPVR are only diagnosed when they have testing done for other reasons.

Partial anomalous pulmonary venous return symptoms

When children have partial anomalous pulmonary venous return symptoms, they may include:

• Shortness of breath
• Frequent respiratory infections
• Slow growth in weight or height
• Fatigue when exercising
• Heart murmur

If PAPVR symptoms don’t start until a person is older, they may include fatigue upon exertion, shortness of breath and frequent respiratory infections as well as:

• Rapid heart rate
• Heart palpitations
• Chest pain

Anomalous pulmonary venous return diagnosis

When you get an ultrasound during your pregnancy, your baby’s heart and its blood vessels are parts that receive careful attention. That’s because the heart is so important to your baby’s health. Because of this, your baby may receive an anomalous pulmonary venous return diagnosis before they’re born. When that happens, it gives doctors more time to evaluate your baby and plan for any treatment.

If a congenital heart defect isn’t diagnosed during pregnancy, your baby’s doctor will determine whether they have a congenital heart disease by performing a physical exam. They’ll listen to your baby’s heart and if they suspect there’s a problem they’ll order noninvasive cardiac imaging such as an echocardiogram, MRI, CT scan or EKG.

Treatment of anomalous pulmonary venous return

Your baby’s health care team will work together to determine your baby’s anomalous pulmonary venous return treatment.

Partial anomalous pulmonary venous return treatment

For many babies with PAPVR, doctors may recommend monitoring them over time to see if they develop symptoms or complications.

When a baby has other heart defects, or if a significant amount of blood is being pumped to the right side of the heart and the right lung (shunted), cardiac surgery may be recommended to relieve serious symptoms and prevent complications like heart failure.

Total anomalous pulmonary venous return treatment

When all your baby’s pulmonary veins are connected to their heart in the wrong places, surgery is required.

Babies need open-heart surgery to repair total pulmonary vein anomalies. Their cardiac surgeon will reconnect the pulmonary veins in the correct places and repair the hole between the left atrium and right atrium (atrial septal defect). Sometimes complications occur, such as blockage of the pulmonary artery at the new connection point, pulmonary high blood pressure or an irregular heartbeat (arrhythmia). These may be treated with surgery or medications.

Surgery for anomalous pulmonary veins is usually successful.

Periodic monitoring

With modern treatment, most babies will lead normal lives. However, all babies and children with anomalous pulmonary veins will need to see a cardiologist for check-ups throughout their lives. Their doctor will ask about potential symptoms, perform testing and check for complications like any blockages in the veins.

At Advocate Children’s Hospital, you’ll also have access to support groups and other resources to help you manage your baby’s care.

Contact us with your questions about heart care for your child.

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