Total Anomalous Pulmonary Venous Return (TAPVR)

Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection (TAPVC), is a rare heart defect in which the blood vessels that drain the lungs (pulmonary veins) are not connected normally to the heart. Instead, the pulmonary veins are redirected abnormally to other chambers of the heart. About 1 in every 20,000 babies is born with TAPVR.

In order to get blood to the body, most babies with TAPVR also have another heart defect, called atrial septal defect, which is a hole from the right atrium to the left atrium.

There are four major types of TAPVR. Each is based on where the pulmonary veins connect to the heart:

• Supracardiac, where the pulmonary veins make an abnormal connection above the heart
• Cardiac, where the pulmonary veins connect behind the heart
• Infracardiac, where the pulmonary veins connect below the heart
• Mixed, a combination of any of the connections above

TAPVR can occur with obstruction, meaning that some of the draining blood vessels are obstructed. This can cause high blood pressure in the lungs (pulmonary hypertension) and can be a surgical emergency.

Children with TAPVR will need surgery in infancy to repair the problem.

Most babies born with total anomalous pulmonary venous return (TAPVR) are very ill soon after birth. Symptoms may include:

• A bluish tint to the skin and lips
• Trouble breathing
• Rapid breathing
• Poor feeding or poor growth

If your child has any of these symptoms, your pediatrician may refer you to a pediatric cardiologist for testing.

In many cases, we don’t know what causes TAPVR. It occurs because of abnormal development of the heart’s pulmonary veins during early fetal growth.

Some congenital heart defects may have a genetic link, causing heart problems to occur more often in certain families. Most often, though, this heart defect occurs by chance, with no clear reason for its development.

In some cases, total anomalous pulmonary venous return is found before birth on a fetal echocardiogram.

Most babies with total anomalous pulmonary venous return (TAPVR) have symptoms on the day they’re born. Severe TAPVR can usually be diagnosed promptly based on symptoms and tests, including chest X-ray and cardiac ultrasound. These babies are generally admitted immediately to the hospital.

Babies with less severe TAPVR may have symptoms in the first few days of life. If your newborn baby is born with a bluish tint to the skin or is having difficulty breathing, you may be referred to a pediatric cardiologist to determine a diagnosis.

Your baby’s doctor may order one or more additional tests to diagnose or confirm a diagnosis of TAPVR:

• Cardiac MRI
• Electrocardiogram (ECG or EKG)
• Cardiac catheterization

Newborns with severe TAPVR will need emergency surgery shortly after birth. They often need to be admitted to the cardiac intensive care unit (CICU) and require intensive support with medications and a ventilator (breathing machine).

Some babies with severe TAPVR may need a specialized life support system called ECMO (extracorporeal membrane oxygenation), an advanced technology that functions as a replacement for a critically ill child's heart and lungs.

Babies with less severe TAPVR usually have surgery in the days or weeks after they're diagnosed.

The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD).

Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving.

Children who have had a repair for TAPVR will require lifelong checkups with a cardiologist to make sure their veins remain open. If the veins become narrowed, they may need catheterizations or surgeries to repair these veins.

Our team in the Boston Children’s Department of Cardiac Surgery treat some of the most complex pediatric heart conditions in the world. Our specialized clinicians can often diagnose this condition during fetal echocardiogram.

Our cardiac surgeons have vast experience in repairing this defect, and work with nurses and doctors who are focused on providing expert care after surgery. Adult patients with TAPVR are followed by Boston Children’s cardiologists with special training for adults with congenital heart problems.