Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect. Most of the time, the cause of this heart problem is unknown.
In this heart condition, the four blood vessels (pulmonary veins) that carry oxygen-rich 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.
This heart defect causes oxygen-rich blood from the lungs to mix with oxygen-poor blood from the body. This keeps oxygen-rich blood from being carried throughout the body. Your child cannot live with total anomalous pulmonary venous return long-term because he or she needs oxygen-rich blood throughout the body.
There are different types of total anomalous pulmonary venous return. The type of TAPVR your child has depends on where the pulmonary veins are connected.
The complications of TAPVR include:
- Enlarged heart
- Lung problems, including respiratory failure and high blood pressure in the lungs
- Abnormal heart rhythms
- Heart failure
- Slowed growth and development
- Enlarged liver
- Need for re-operation
Other heart defects often occur with TAPVR. Sometimes, these other heart conditions actually help a child have enough oxygen in their blood until they can have surgery. These other congenital heart defect include:
- Atrial septal defect. In this heart condition, there is an opening between the two upper chambers of the heart. This causes oxygen-poor and oxygen-rich blood to be mixed.
- Patent ductus arteriosus. The ductus arteriosus is a blood vessel that carries blood away from the heart in a developing baby. It usually closes soon after birth. If it stays open, it’s called patent ductus arteriosus. Sometimes it stays open with TAPVR. This causes oxygen-poor and oxygen-rich blood to be mixed.
- Since ventricle. In this heart condition, there is only one ventricle instead of the normal two.
Total Anomalous Pulmonary Venous Return Symptoms
The symptoms of total anomalous pulmonary venous return may be similar to symptoms of other conditions. Your child’s healthcare provider must diagnose him or her with this condition.
Newborns with total anomalous pulmonary venous return will have cyanosis - blue coloring of their skin, lips, and nailbeds. 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 of TAPVR can occur a bit differently in each child. They can include:
- Fast or troubled breathing
- Fast heart rate
- Cool, clammy skin
- Tiredness and little movement
- Poor feeding
- Poor pulses
- Enlarged liver
- Heart murmur
Diagnosis of Total Anomalous Pulmonary Venous Return
A healthcare provider may first spot total anomalous pulmonary venous return 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 and can include:
- Chest X-ray. A chest X-ray may show changes in the heart and lungs caused by total anomalous pulmonary venous return.
- Electrocardiogram (ECG). 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.
- Echocardiogram (echo). This test uses sound waves to produce a moving picture of the heart and heart valves. An echo can show the structural changes in the heart associated with total anomalous pulmonary venous return.
- Cardiac catheterization. A cardiac catheterization gives detailed information about the structures inside the heart. In this test, a small, thin, flexible tube 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 blood pressure and oxygen levels will be checked during the procedure.
- Computed tomographic imaging (CT) and magnetic resonance angiography (MRA). A CT and MRA can provide detailed information about the pathways and connections of the pulmonary veins.
Treatment of Total Anomalous Pulmonary Venous Return
Treatment of TAPVR 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. Total anomalous pulmonary venous return may also be treated through medical management and cardiac catheterization.
- Medical management. At first, your baby may get the following care:
- Ventilator. Supplemental oxygen or a machine that allows breathable air to move in an out of the lungs (ventilator). This helps with breathing.
- Prostaglandin therapy. This medicine is used to keep the ductus arteriosus open and allow blood to flow through the heart. This medicine isn’t used in all types of TAPVR.
- Medication. Medicine can support the function of the heart
- Mechanical ventilation. A breathing machine may be needed to help increase oxygen levels and remove carbon dioxide from the child's lung, usually through the mouth and into the windpipe (trachea).
- Extracorporeal membrane oxygenation (ECMO). This technique provides both the cardiac and respiratory support in infants who are not able to maintain oxygen levels on mechanical ventilation.
- Surgery. The surgery for total anomalous pulmonary venous return is done through an incision through the breastbone 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 severity of the defect your baby has. Your child’s heart doctor will explain the procedure to you in detail.
- Cardiac catheterization. This procedure can be used to diagnose and treat TAPVR. Your child’s healthcare provider may do a test called balloon atrial septostomy before the cardiac catheterization. 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.
Living with TAPVR
Your child will likely stay in the hospital until the total anomalous pulmonary venous return condition 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 a heart doctor for regular checkups for the rest of his or her life.
Your child may also need the following care:
- As children grow, they may need more heart repair procedures.
- Your child's heart doctor may give your child antibiotics before surgeries or dental procedures. This is to prevent infections. But this often isn’t needed.
- Some children are delayed in their development. Talk to your child’s healthcare provider about his or her development. Your child may need to be checked for issues.