Total Anomalous Pulmonary Venous Return

What is total anomalous pulmonary venous return?

Total anomalous pulmonary venous return (TAPVR) is a birth defect in which the lung veins do not attach to the left atrium. Instead, they attach to the right atrium or to the blood vessels leading into the right atrium.

Normally, 4 veins come from the lungs (2 from the left lung and 2 from the right lung). They carry oxygen-rich (red) blood into the right atrium. Then the blood flows into the left ventricle, which pumps it out to the body.

After delivering oxygen around the body, the blood returns to the heart and goes into the right atrium. Then it flows into the right ventricle, which pumps it to the lungs to pick up oxygen.

In TAPVR, the 4 lung veins usually join to form 1 common vein. Instead of connecting to the left atrium, the common vein connects to the right atrium or to the vessels that bring oxygen-poor (blue) blood from the body into the right atrium.

Sometimes, the 4 lung veins may take different routes to connect to the right atrium or to the vessels that bring oxygen-poor blood from the body into the right atrium. This is called mixed TAPVR. In either case, most of the blood flows only between the right side of the heart and the lungs, not out to the rest of the body.

Babies with TAPVR get some blood to their body because they have a hole in the septum between the upper chambers of the heart, called an atrial septal defect or ASD. When oxygen-poor blood from the body and oxygen-rich blood from the lungs mix in the right atrium, some of this blood flows through the hole into the left atrium. Then the blood flows into the left ventricle, which pumps it out to the body.

  • With this condition, the baby’s body does not get as much oxygen as it should.

    Their right ventricle and pulmonary artery (the artery that carries blood from the right ventricle to the lungs) will both become larger than normal because they are handling too much blood.

    In some babies, the lung veins are blocked, which causes more severe problems with blood flow and breathing.

Total Anomalous Pulmonary Venous Return at Seattle Children’s

  • U.S. News & World Report consistently ranks Seattle Children’s cardiology and heart surgery program as one of the best in the country. With more than 40 pediatric cardiologists, we have experience diagnosing and treating every kind of heart problem.

    Our heart team has treated many children with total anomalous pulmonary venous return. We have extensive experience with the surgery these patients require. We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit to help care for children who undergo heart surgery.

    See our statistics and outcomes for total anomalous pulmonary venous return repair.

    The Adult Congenital Heart Disease Program shared by Seattle Children’s and the University of Washington can help with care throughout your child’s life.

    Seattle Children’s has been treating children since 1907. Our team members are experts in their fields and in meeting the unique needs of children. For example, the doctors who give your child anesthesia (sedation) are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialistsknow how to help children understand their illnesses and treatments in ways that make sense for their age.

  • When you come to Seattle Children’s, a team of people will take care of your child. Along with your child’s heart doctor (cardiologist), cardiac surgeons, cardiac anesthesiologists, cardiac intensive care specialists, newborn specialists (neonatologists), lung doctors (pulmonologists), advanced nurse practitioners, nurses, child life specialists, social workers and others will care for your child, if their help is needed.

    We work together to meet all of your child’s health needs and help your family through this experience. We’ll discuss treatments in ways you can understand and involve you in every decision.

    Read more about the supportive care we offer.

Symptoms of Total Anomalous Pulmonary Venous Return

Most babies with this condition start to have symptoms soon after birth. They may have symptoms like these: 

  • Fast breathing or working hard to breathe
  • Being more tired than normal
  • Trouble feeding
  • Not growing as fast as normal
  • Bluish skin, lips or nailbeds (cyanosis) 

Some children do not start having symptoms until later in infancy.

Diagnosing Total Anomalous Pulmonary Venous Return

To diagnose this condition, your doctor will examine your child and use a stethoscope to listen to their heart.

The doctor will ask for details about your child’s symptoms, their health history and your family health history.

Your child will also need tests that provide information about how their heart looks and works. These may include: 

Treating Total Anomalous Pulmonary Venous Return

This condition can only be repaired by surgery.

In the operation, the doctor opens the back of the left atrium and attaches the common vein to the wall of the left atrium. This creates proper flow of oxygen-rich blood from the lungs to the heart. The doctor also closes the abnormal connection that went to the right side of the heart, as well as the atrial septal defect.

The timing of the surgery depends on how severe your child’s condition is. Some children need surgery soon after birth because they have severe symptoms. Sometimes cardiac catheterization and placement of a stent in the common vein is used to help stabilize babies whose common vein is blocked.

If your child’s symptoms are not severe, your doctor may suggest waiting so your child can grow. This makes it easier to do the surgery. Most children have surgery within the first 6 months of life.

To meet your child’s long-term healthcare needs, we have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready.

Contact Us 

Contact the Heart Center at 206-987-2515 to request an appointment, a second opinion or more information.

Providers, see how to refer a patient.