Total Anomalous Pulmonary Venous Return  (TAPVR)

What is total anomalous pulmonary venous return?

Total anomalous pulmonary venous return (TAPVR) is a rare heart problem where the pulmonary veins do not connect to the heart the way they should. TAPVR is a type of congenital heart disease. This means it’s a heart condition that a baby is born with. There are several different types of TAPVR, depending on where the veins connect.

Normally, the pulmonary veins carry oxygen-rich blood from the lungs to the upper left chamber of the heart, called the left atrium. The heart then pumps out this oxygen-rich blood to the rest of the body. In children with TAPVR, the pulmonary veins carry blood into the right atrium instead of the left. This change in flow causes oxygen-poor blood to mix with the oxygen-rich blood, and the baby’s body doesn’t get as much oxygen as it should.

Babies born with TAPVR also have an atrial septal defect (ASD). This is a hole between the two upper heart chambers. The ASD allows some of the oxygen-rich to be pumped out to the body. Some children with TAPVR may also have other holes in their heart or other congenital heart conditions.

TAPVR is a serious heart problem. Babies born with TAPVR will need at least one surgery to restore correct blood flow in the heart.

What are the signs and symptoms of TAPVR?

Babies born with TAPVR usually have symptoms shortly after birth. Symptoms TAPVR in infants may include:

• Blue or gray tint to the skin, nails, or lips
• Trouble breathing or shortness of breath
• Trouble feeding
• Lack of energy
• Heart murmur

What causes TAPVR?

Experts are not sure what causes TAPVR.

How is TAPVR treated?

All babies born with TAPVR need surgery to treat the condition and restore normal blood flow through the heart. Some babies will need emergency surgery soon after birth, while others might not have surgery for a few weeks. During the surgery, the surgeon will connect the pulmonary veins to the left atrium, close off any abnormal connections between the veins, and close the ASD.

Nationally ranked in pediatric cardiology and cardiac surgery, the Arkansas Children's Heart Institute offers world-class expertise and state-of-the-art technology to infants, children, adolescents and young adults. Your pediatric cardiology team at Arkansas Children’s is experienced in treating TAPVR and will work with you to determine the best course of treatment for your child.

What about emotional and psychological support?

When your child is born with a medical condition like TAPVR, you and your family may experience a range of emotions, including anxiety, fear, anger, and frustration. If you or your child ever need extra support, our social workers are here to help guide your family through the emotional challenges of living with an illness. They can help connect you with resources and referrals for counseling, support groups, and other types of help. And if your toddler or child needs extra support during clinic visits or procedures, our team of child life specialists can offer education, play, and emotional support to help them cope and better understand their healthcare journey.