Total Anomalous Pulmonary Venous Connection (TAPVC) | Heart & Vascular | Loyola Medicine

Total Anomalous Pulmonary Venous Connection (TAPVC)

Overview and Facts about Total Anomalous Pulmonary Venous Connection (TAPVC)

Total anomalous pulmonary venous connection (TAPVC) is a rare infant heart defect in which all four of the pulmonary veins are in the wrong position, causing abnormal blood flow. There are three types of TAPVC:

  • Supercardiac: This is where the blood drains into one of the brachiocephalic veins (the two large veins that are positioned on either side of the neck), or into the superior vena cava.
  • Cardiac: This is where the blood drains into the right atrium (upper chamber) or the coronary sinus (a vessel that collects blood from the heart muscle).
  • Infradiaphragmatic: This is where blood drains in the hepatic veins (veins that transport blood from the gallbladder, spleen, GI tract, and pancreas to the liver).

TAPVC can also have an obstruction. This happens when the mispositioned vein enters a blood vessel at a sharp angle. It can cause high blood pressure and poor blood oxygen flow because blood is unable to enter the vein easily.  

Signs and Symptoms of Total Anomalous Pulmonary Venous Connection (TAPVC)

Signs and symptoms of TAPVC include:

  • Pounding heart
  • Difficulty breathing
  • Bluish skin
  • Weak pulse
  • Poor appetite
  • Extreme fatigue

Causes and Risk Factors of Total Anomalous Pulmonary Venous Connection (TAPVC)

The cause of this type of heart condition is typically not known. However, in some cases, it is thought to be due to changes in an infant’s genes or chromosomes. Other contributing risk factors include the mother coming into contact with environmental toxins, what she eats and drinks, and medicines she is taking during pregnancy.

Tests and Diagnosis of Total Anomalous Pulmonary Venous Connection (TAPVC)

TAPVC may be diagnosed during pregnancy after the mother has undergone prenatal screening tests. It may also be diagnosed during an ultrasound scan. If the health care provider suspects the baby has TAPVC, a fetal echocardiogram will confirm the diagnosis. This test creates an image of the baby’s heart and blood vessels.

However, TAPVC is seldom diagnosed during pregnancy. This is because it is difficult for doctors to see the pulmonary veins on prenatal tests since very little blood goes through the lungs before the baby is born.  

If an infant is born with TAPVC, symptoms will usually occur at birth or very soon after. The physician will use a stethoscope to examine the infant’s heartbeat. A heart murmur may be present. If the physician suspects a problem, an echocardiogram will confirm the diagnosis. An electrocardiogram (EKG), which measures the heart’s electrical activity, may also be used.

Treatment and Care for Total Anomalous Pulmonary Venous Connection (TAPVC)

If TAPVC occurs without an obstruction, corrective surgery can be done within the first month of the infant’s life. During the procedure, the four pulmonary veins are reconnected to the proper positions and any other defects are closed. If TAPVC occurs with an obstruction, emergency surgery is usually performed to allow normal circulation.