Total Anomalous Pulmonary Venous Return

What is a Pediatric Total Anomalous Pulmonary Venous Return?

Total anomalous pulmonary venous return (TAPVR) makes it hard for the heart to pump oxygen-rich blood to the rest of the body. In a healthy heart, the right-side pumps blood to the lungs to get oxygen. That blood then returns through the pulmonary veins to the left side of the heart to be pumped to the body. TAPVR develops during the first four to six weeks of pregnancy because the child's heart doesn’t develop normally. In TAPVR, a child's blood vessels (pulmonary veins) connect to the wrong side of the heart. When this happens, the oxygen in the lungs can't be brought to the rest of the body efficiently.

What are the different types of Pediatric Total Anomalous Pulmonary Venous Return?

There are three types of TAPVR.

Supracardiac Total Anomalous Pulmonary Venous Return

This is the most common type of TAPVR. The word “supra” means “above or over.” In supracardiac TAPVR, the pulmonary veins come together and connect to a blood vessel called the superior vena cava above the heart. The superior vena cava usually brings oxygen-poor blood from the upper part of the body to the heart so it can be pumped to the lungs where it picks up oxygen. In supracardiac TAPVR, this abnormal connection reduces the delivery of oxygen-rich blood to the body.

Cardiac Total Anomalous Pulmonary Venous Return

There are two types of cardiac TAPVR. In both types, the oxygen-rich blood returning from the lungs is connected to the wrong side of the heart:

• Pulmonary veins connect to the upper right heart chamber (atrium).
• Pulmonary veins connect to the coronary sinus. The coronary sinus brings oxygen-poor blood from the heart muscle into the right heart chamber.

Both of these conditions decrease delivery of oxygen-rich blood to the body.

Infracardiac Total Anomalous Pulmonary Venous Return

In a typical heart, the inferior vena cava is the main blood vessel that brings oxygen-poor blood from the bottom part of the body to the heart. With infracardiac TAPVR, the pulmonary veins don’t form correctly and connect to the inferior vena cava to the liver blood vessels below the heart. The pulmonary veins may be narrowed in this type of TAPVR. This condition can cause a severe decrease in the delivery of oxygen-rich blood to the body.

What are the signs and symptoms of a Pediatric Total Anomalous Pulmonary Venous Return?

In some cases, signs of TAPVR show up during prenatal tests, such as a fetal ultrasound. Children can develop symptoms within the first weeks, months or year of their life. These symptoms include:

• Skin that appears ashen or blue from poor circulation (also called cyanosis)
• Rapid breathing or shortness of breath
• Heart murmur
• Weak pulse
• Difficulty feeding
• Rapid heartbeat

How is a Pediatric Total Anomalous Pulmonary Venous Return diagnosed?

Cardiologists use several methods and tests to diagnose TAPVR. These include:

• Medical history, where we’ll ask you about your child’s medical history and your family’s medical history.
• Physical examination, where we will examine your baby’s heart function.
• Pulse oximetry screening, to measure the oxygen levels in your child’s blood.
• Electrocardiogram (EKG), to record the electrical signal from your child’s heart.
• Echocardiogram, which is an ultrasound exam of the heart.
• CT scan, which takes a detailed image of the heart and may be needed to help us confirm the diagnosis.

What causes a Pediatric Total Anomalous Pulmonary Venous Return?

We don’t know what causes TAPVR, but we do know the defect develops during the first four to six weeks of pregnancy. It’s not caused by anything a mother or father did or did not do.

How is a Pediatric Total Anomalous Pulmonary Venous Return treated?

Children with TAPVR need surgery to restore normal blood flow to and through the heart. Your child’s surgeon will discuss which surgery is right for your child.

Exactly when your child has surgery depends on how serious their condition is:

• If your child is very sick (critically ill) they will have surgery right away.
• Children who are not critically ill may wait two to six weeks for surgery, depending on the structure of the abnormal connections, as well as other symptoms.

After surgery, your child will need regular follow-up care to check for complications and other heart conditions.