Partial anomalous pulmonary venous return

Overview

Partial anomalous pulmonary venous return is a rare heart problem that's present at birth. That means it is a congenital heart defect.

Other names for this condition are:

• PAPVR.
• Partial anomalous pulmonary venous connection.
• PAPVC.

In this condition, some of the blood vessels of the lungs attach to the wrong place in the heart. These blood vessels are called the pulmonary veins.

In a typical heart, oxygen-rich blood goes from the lungs to the upper left heart chamber, called the left atrium. Then the blood flows through the body.

In PAPVR, blood flows from the lungs into the upper right heart chamber, called the right atrium. Extra blood flows to the right side of the heart. This may cause swelling of the right heart chambers.

Some people with PAPVR have a hole between the upper heart chambers called an atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. A child born with Turner syndrome has an increased risk of PAPVR.

Symptoms

Symptoms depend on whether there are other heart problems. A common symptom of PAPVR is breathing trouble.

Diagnosis

If PAPVR occurs with other heart problems, it may be diagnosed soon after birth. If the condition is mild, it may not be diagnosed until adulthood.

A health care provider does a physical exam and listens to the heart with a stethoscope. A whooshing sound, called a heart murmur, may be heard.

An echocardiogram is done to diagnose partial anomalous pulmonary venous return. This test uses sound waves to create images of the beating heart. An echocardiogram shows the pulmonary veins and the size of the heart chambers. It also measures the speed of blood flow. An echocardiogram can help diagnose a hole in the heart.

Other tests such as an electrocardiogram (ECG or EKG), a chest X-ray or a CT scan may be done if more information is needed.

Treatment

Surgery to repair the heart may be needed if:

• A lot of oxygen-rich and oxygen-poor blood mixes in the heart.
• The condition causes many lung infections.

If you don't have symptoms, surgery may not be needed. If surgery for another heart condition is needed, surgeons may repair PAPVR at the same time.

During PAPVR repair surgery, the heart surgeon:

• Reconnects the pulmonary veins to the left upper heart chamber.
• Closes any holes in the heart.

A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It's best to see a health care provider who is trained in congenital heart diseases. This type of provider is called a congenital cardiologist.