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Total anomalous pulmonary venous return (TAPVR) is a critical birth defect of the pulmonary veins that causes oxygen-rich blood from the lungs to return to the wrong side of the heart.
Normally, our pulmonary veins return to the left atrium. In TAPVR, the veins return elsewhere, like the superior vena cava (supracardiac), inferior vena cava (infracardiac), the right atrium (intracardaic), or even a combination of the above or to other vessels. This 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 have varying degrees of cyanosis associated with TAPVR, depending on the communication between the chambers of the heart — usually an atrial septal defect. 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 likely will need surgery in the first year of life.
As the leading providers of pediatric heart care in Louisville and Southern Indiana, the board-certified and fellowship-trained cardiothoracic surgeons at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, 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.
Symptoms of TAPVR usually include:
Other signs may include a heart murmur — or whooshing sound — that a doctor can hear through a stethoscope. Mild cases may not be noticed for weeks or months until the child has symptoms such as tiring easily, rapid or difficult breathing, or poor growth.
TAPVR is a rare heart defect, and in most cases the cause is unknown. While genetics can raise the risk, the congenital defect most often happens by chance.
Ultrasound or other prenatal tests can reveal the condition during pregnancy. If your doctor suspects your baby has TAPVR, he or she will order additional tests, such as a fetal echocardiogram. A fetal echocardiogram is an ultrasound of the baby’s heart and major blood vessels before birth. Norton Children’s Heart Institute physicians will assist in diagnosis and help prepare a delivery plan for both mom and baby, including the baby’s immediate care plan following delivery.
TAPVR also can be found after a baby is born. If an infant shows signs of a heart defect, an echocardiogramcan be performed to help diagnose TAPVR.
Babies born with TAPVR will need surgery to restore normal blood flow through the heart. Surgery options include placing the child on a heart-lung machine (cardiopulmonary bypass) and making the connection between the pulmonary veins and the left atrium.
In the most common type of TAPVR, all four pulmonary veins come to a central spot — called a confluence — that is behind the left atrium. In this case, a full repair is done by connecting the confluence into the back of the left atrium. In other forms, the surgeons find other ways to connect the pulmonary veins back to the left atrium.
After surgery, it is possible that scar tissue can form around the connection between the pulmonary veins and the left atrium. If the scar tissue growth is severe enough, additional surgery may be needed. Between 15% and 20% of children with TAPVR may need cardiac catheterization or surgery later in life to repair narrowed veins.
A baby who has an obstructive form of TAPVR (meaning the blood cannot get to the heart from the lungs) may need emergency surgery. This surgery often is performed when the baby is just hours old. If the pulmonary veins are not obstructed, surgery can wait until a few weeks after birth.
Surgery recovery is closely related to how healthy the lungs are before surgery. Approximately 5% to 8% of children who have the procedure do not survive. The risk varies greatly from child to child. It depends on what kind of TAPVR the child has and the amount of obstruction the child has before surgery.
Children with surgically repaired TAPVR may have lifelong complications. They may need medication to prevent infection of the heart’s lining (endocarditis) or an irregular heartbeat (arrhythmia). Children and adults with the condition will need regular visits with a pediatric cardiologist to monitor their progress and complications, as well as screen for other conditions that could develop over time, including erratic heartbeat, blood clots, leaky valves and blocked blood vessels.