TAPVR Repair

Total anomalous pulmonary venous return (TAPVR) is a rare, congenital (present from birth) heart condition. TAPVR repair requires surgery.

Normally, the pulmonary veins return to the heart’s left atrium. In TAPVR, the veins return elsewhere, such as the superior vena cava (supracardiac), inferior vena cava (infracardiac), right atrium (intracardaic), or even a combination of the above or to other vessels.

This condition causes oxygen-rich blood to mix with oxygen-poor blood in the right atrium. The mixed blood gets to the body through a hole between the heart chambers and robs the body of the oxygen it needs.

Children with TAPVR will have varying degrees of cyanosis — a blue discoloration seen in the lips, hands and feet. This depends on the communication between the chambers of the heart, usually being an atrial septal defect (ASD).

Children can be extremely sick if there is an obstruction to blood flow in TAPVR. Children with obstructed blood flow will need surgery immediately. All babies born with TAPVR will likely need surgery in the first year of life.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.

As the leading providers of pediatric heart care in Louisville and Southern Indiana, our cardiothoracic surgeons are experienced with successfully repairing TAPVR. They have the skills and training to know what needs to be done quickly to repair TAPVR — sometimes in the first hours of a baby’s life.

TAPVR Repair Requires Surgery

The most severe cases of TAPVR occur when the baby’s pulmonary veins are obstructed. This causes blood to back up into the lungs. The cardiothoracic surgeons at Norton Children’s Heart Institute may have to perform emergency surgery in cases of obstructed TAPVR. Even without obstructions, babies typically have TAPVR repair surgery days or weeks after diagnosis.

Your child will be sedated (asleep) in the care of our pediatric cardiac anesthesiologists. During the open heart procedure, the board-certified and fellowship-trained specialists at Norton Children’s Heart Institute will move the pulmonary veins and connect them to the back of the left atrium. This will create a normal blood flow pattern to the entire body.

With the normal blood flow in place, children who have had a TAPVR repair at Norton Children’s Heart Institute can expect to grow up with few, if any, symptoms.

Why Choose Norton Children’s Heart Institute

The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Institute’s Adult Congenital Heart Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
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