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Pediatric Total Anomalous Pulmonary Venous Return (TAPVR)

What is total anomalous pulmonary venous return?

Anatomy of the heart, normal

Total anomalous pulmonary venous return (TAPVR) is a congenital (present at birth) heart defect. Due to abnormal development of the prenatal heart during the first eight weeks of pregnancy, the vessels that bring oxygen-rich (red) blood back to the heart from the lungs are improperly connected.

Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs through four pulmonary veins. It then passes into the left ventricle, and is pumped through the aorta out to the body.

In TAPVR, the four pulmonary veins are connected somewhere besides the left atrium. There are several possible places where the pulmonary veins can connect. The most common connection is to a blood vessel that brings oxygen-poor (blue) blood back to the right atrium, usually the superior vena cava.

Anatomy of a heart with total anomalous pulmonary venous return

In TAPVR, oxygen-rich (red) blood that should return to the left atrium, the left ventricle, the aorta and then the body, instead mixes with the oxygen-poor (blue) blood flowing into the right side of the heart. This situation by itself will not support life, because there is no way for oxygen-rich (red) blood to be delivered to the body.

Other heart defects are often associated with TAPVR and they actually help the infant with total anomalous pulmonary venous return to live until surgical intervention is possible:

  • An opening in the atrial septum will allow blood from one side to mix with blood from another, creating "purple" blood with an oxygen level somewhere in-between that of the oxygen-poor (blue) and the oxygen-rich (red) blood.

  • Patent ductus arteriosus will also allow mixing of oxygen-poor (blue) and oxygen-rich (red) blood through the connection between the aorta and pulmonary artery. The "purple" blood that results from this mixing is beneficial, providing at least a little oxygen to the body.

Total anomalous pulmonary venous return occurs equally in boys and in girls.

Prevention and Risk Assessment

Diagnosis

Treatment

Children's Team

Children's Team

Providers

Richard Jonas

Richard Jonas

Division Chief, Cardiac Surgery
Co-Director, Children's National Heart Institute
Departments

Departments

Cardiac Imaging

We have expertise in the full spectrum of cardiac imaging, including transesophageal, prenatal, 3-D, intracardiac, and stress echocardiography and cardiac MRI.

Cardiac Surgery

Our pediatric heart surgery team performs twice the number of surgeries of any other hospital in the region, with some of the best outcomes in the nation.

Prenatal Cardiology Program

Our heart care begins before babies are even born, in our dedicated Prenatal Cardiology Program, where we diagnose and even treat prenatal heart conditions.

Cardiology

The pediatric heart experts at Children’s National Hospital in Washington, D.C., provide advanced care for unborn babies, children and young adults with heart conditions.

Children's National Heart Institute

Our expert pediatric heart team, including more than 40 subspecialties, offer advanced heart care and excellent outcomes for thousands of children every year.

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