In this condition, the 4 blood vessels (pulmonary veins) that carry oxygen-rich (red) blood to the heart from the lungs aren’t connected right. They should be connected to left chamber (atrium) of the heart. Instead, they’re connected somewhere else.
There are different types of TAPVR. The type your child has depends on where the pulmonary veins are connected.
This condition causes oxygen-rich blood from the lungs to mix with oxygen-poor (blue) blood from the body. This keeps oxygen-rich blood from being carried throughout the body. Your child cannot live with TAPVR long-term because he or she needs oxygen-rich blood throughout the body.
Other heart defects often occur with TAPVR. They actually help infants have enough oxygen in their blood until they can have surgery. These other defects include:
The symptoms of TAPVR may be similar to symptoms of other conditions. Your child’s healthcare provider must diagnose him or her with this condition.
Newborns with TAPVR will have blue coloring of their skin, lips, and nailbeds (cyanosis). This happens in the first hours or days of their life. How severe your child’s condition is depends on how much blood is able to get to his or her body.
Other symptoms can occur a bit differently in each child. They can include:
A healthcare provider may first spot TAPVR in a baby during an ultrasound in pregnancy. After birth, if your child has signs of TAPVR, a pediatric cardiologist or neonatologist will check him or her. A pediatric cardiologist is a doctor with special training to diagnose and treat heart problems in babies and children. A neonatologist is a doctor with special training to diagnose and treat problems in newborns. These include babies born premature and full-term.
Next, your child’s doctor may do tests to check for heart problems. The tests your child needs depends on his or her age and condition. They also depend on the doctor’s preferences.
A chest X-ray may show changes in the heart and lungs caused by TAPVR.
An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and detects heart muscle stress. These problems may be caused by TAPVR.
This test uses sound waves to produce a moving picture of the heart and heart valves. An echo can show the structural changes of TAPVR.
A cardiac catheterization gives detailed information about the structures inside the heart. In this test, a small, thin, flexible tube (catheter) is put into a blood vessel in your child’s groin. Then the healthcare provider guides it to your child’s heart. There, he or she will inject your child with contrast dye to see the heart more clearly. Your child’s healthcare provider will give him or her medicine to help relax and prevent pain (sedation). Your child’s blood pressure and oxygen levels will be checked during the procedure. Your child may not need this test if a diagnosis is made during the echo.
A CT and MRA can provide detailed information about the pathways and connections of the pulmonary veins.
Treatment will depend on your child’s symptoms, age, and overall health. It will also depend on how severe the condition is.
All children with a TAPVR will need to have surgery to fix it. Your baby may need to go into the intensive care unit (ICU) for treatment. TAPVR may also be treated through medical management and cardiac catheterization.
At first, your baby may get the following care:
This procedure can be used to diagnose and treat some heart defects. To treat TAPVR, your child’s healthcare provider may do a test called balloon atrial septostomy. This test makes it easier for oxygen-rich blood to get to the left side of the heart and then the rest of the body.
The surgery is done through a cut (incision) through the breastbone (sternum) and into the chest. Your baby will be connected to a heart-lung machine during surgery. This device does the work of your baby's heart and lungs during surgery. The details of the surgery depend on the type of defect your baby has. Your child’s heart doctor will explain the procedure to you.
Your child will likely stay in the hospital until TAPVR is repaired. Your child’s healthcare team will give you instructions about special treatments or medicines your child needs before your child leaves the hospital. For instance, your child may need special formula and supplemental feedings to help with his or her growth. Your child’s healthcare team will also help you plan for home healthcare if you need it.
Many children with TAPVR will grow and develop normally after they have surgery. Talk with your child's cardiologist about how much and what kinds of physical activity your child can safely do. Your child will still need to see his or her heart doctor for regular checkups for the rest of his or her life. Your child’s follow-up care should be at a center that specializes in congenital heart defects. Ask your child's healthcare provider about your child’s outlook.
Your child may also need the following care:
Tips to help you get the most from a visit to your child’s healthcare provider:
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