Total Anomalous Pulmonary Venous Connection

If your child has total anomalous pulmonary venous connection, our pediatric cardiology specialists can help. We have the experience and expertise to provide your child an accurate diagnosis and personalized treatment plan, all in the safe and intimate space of our Children’s Heart Center.

We support you and your family with compassion throughout the process of diagnosis and treatment. As we explain what is going on with your child, we help you understand your child’s condition and know  that your child is in the right hands. 

About Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), disrupts the normal flow of blood between your baby’s lungs and heart.

Normally, the heart pumps blood from the right lower chamber (the right ventricle) to the pulmonary artery and then to the lungs, where it gains oxygen. The oxygenated blood returns to the heart through four pulmonary veins. The blood goes to the left atrium (the left upper chamber). And then goes to the left lower chamber (the left ventricle) and on to the body.

However, with TAPVC, your baby’s pulmonary veins connect instead to other veins in your baby’s chest or belly. This means the oxygenated blood from the lungs mixes with oxygen-poor blood from these other veins, which then goes back to the right upper chamber of the heart.

When the mixed blood returns to the heart, some of it goes across a hole between the upper chambers of the heart, through an atrial septal defect (ASD). It gets into the left side of the heart and out to the body. This causes low oxygen levels in the body. In addition, the right side of your baby’s heart may be overworked from pumping extra blood.

The abnormally connecting pulmonary veins can go to other veins in the chest above the heart (supracardiac), to veins within the heart (intracardiac), or to veins in the belly (infracardiac). If the abnormal connections between the pulmonary veins and the other veins are obstructed,  blood may back up in your baby’s lungs.

We will explain your baby’s specific condition to you along with how we can help your baby.

Symptoms

Babies born with total anomalous pulmonary venous may become unwell soon after birth, often developing the following symptoms:

  • Blueish or dusky tint to lips, skin, and nails
  • Rapid breathing or working harder while breathing, especially while feeding 

Diagnosis

The diagnosis of this condition most often occurs after birth, but occasionally is made during pregnancy. To diagnose TAPVC in your child, we need to perform some of the following tests:

  • Echocardiogram— a safe, noninvasive procedure that uses high frequency ultrasound to show the structure of your child’s heart, how blood flows through the heart, and overall how your child’s heart and circulatory system are working
  • Pulse oximetry—a noninvasive way to monitor the oxygen content of the blood
  • Cardiac magnetic resonance imaging (MRI)—uses a powerful magnetic field, radio waves, and a computer to produce detailed pictures of the heart and blood vessels to understand the anatomy of veins in complex cases of TAPVR
  • Cardiac computed tomography (CT)—uses X-rays to create detailed images of the blood vessels in complex cases of TAPVR to help us better understand the anatomy of the veins
  • Electrocardiogram (ECG or EKG)—a diagnostic test to measure electrical activity, which displays the heart’s chambers and how your child’s heart is beating
  • Chest X-ray—an image that  shows the position and size of your child’s heart 

Treatments We Offer

The only way to correct TAPVC is with an open-heart operation. Our pediatric cardiac surgeon re-connects your child’s pulmonary veins to the left top chamber of the heart (the left atrium), and repairs the ASD as needed so the heart can function properly.

For children with obstructed TAPVC, we do this repair  as soon as possible. For cases that are not obstructed, your cardiac care team will confer with you to decide on the optimal timing for repair.

Following this important surgery, your child will need to continue to see a pediatric cardiologist throughout childhood and into adulthood

At Mount Sinai, we here for you, your child, and your family for life.

Our Alliance with Children’s Hospital of Philadelphia

Children’s Heart Center—an alliance between Mount Sinai and Children’s Hospital of Philadelphia (CHOP)—ensures that your child has access to the newest diagnostics and treatments for total anomalous pulmonary venous connection.

This alliance combines the skills, knowledge  and compassion of our doctors with that of CHOP doctors, one of the nation’s leading children’s hospitals, according to U.S. News & World Report. 

Why Choose Children’s Heart Center

Our doctors and staff specialize in providing  outstanding personalized care for your child with congenital heart disease, such as total anomalous pulmonary venous connection. We have the expertise to diagnosis and treat your baby’s heart condition using effective technique.

We go the extra mile to give your child the best care possible.