Total Anomalous Pulmonary Venous Return Repair

Total Anomalous Pulmonary Venous Return (TAVPR) is a type of congenital heart defect (present at birth) that affects the pulmonary veins. Arteries carry blood away from the heart; veins carry it toward the heart. The pulmonary veins are very important: They carry the “red” blood that has picked up oxygen in the lungs back to the heart to be pumped out to the body. There are typically four pulmonary veins, two from each lung. Normally, they all connect directly into the heart’s left atrium (left upper chamber).

In TAPVR, there is a problem in the normal connection between the blood vessels coming from the lungs to the left atrium. This problem can take many different forms. Regardless of form, your doctor will typically advise for your baby to get surgery immediately. Surgery is performed via open-heart surgery (involves dividing the breast bone / general anesthesia).

What to Expect Before the Procedure

Before the procedure, your doctor may perform a variety of diagnostic tests, including:

• Echocardiogram (ECHO)
• Computed tomography (CT scan)
• Magnetic resonance imaging (MRI)
• X-Rays
• Electrocardiogram (EKG/ECG)

What to Expect During the Procedure

This procedure usually takes 5 hours to complete, but preparation and recovery may add several hours. The procedure is usually performed in the cardiothoracic operating room (OR). In general, during this procedure:

• Your baby will be sedated, then your doctor will insert a breathing tube through your baby’s throat into their lungs and connect it to a ventilator. This will breathe for your baby during surgery.
• Your doctor will administer general anesthesia (will make your baby feel sleepy).
• The procedure begins when your doctor exposes your heart by dividing the breastbone (sternum) in half. Your doctor then spreads both halves to gain access to your heart (open-heart surgery).
• For this type of surgery, the heart must be still. Prior to doing so, your doctor will place tubes into your baby’s heart so that blood can be pumped through your baby’s body by use of a heart-lung machine. This machine takes over for the heart by replacing the heart’s pumping action and the lungs by adding oxygen to the blood.
• Once the blood has been diverted into the bypass machine for pumping, your doctor will then stop the heart by injecting it with a cold solution.
• When the heart has been stopped, your doctor will start with the procedure.
• In some cases, the surgeon will create a connection between the vessel coming from the lung and the left atrium. The surgeon may close off the vessel where the vessel from the lung was previously joined.
• In other cases, the surgeon will rebuild the wall between the left and right atrium. That way, the blood from the lungs flows directly into the left atrium.
• The surgeon will make other repairs to the heart as needed.
• After the procedure is completed, the doctor will closely check to make sure everything is working properly. Once checked, the doctor will let the blood circulating through the bypass machine back into your baby’s heart.
• Once the procedure is complete, the machine will be turned off. The tubes will be removed and the sternum will be sewn together with the use or sutures or surgical staples.

What to Expect After the Procedure

After the procedure, your baby will be taken to the cardiothoracic intensive recovery unit (CTICU) for a few days. Your baby will also spend several days in the recovery unit. During this time the cardiac team will:

General Guidelines

• Make sure your baby’s vital signs, such as heart rate and breathing, are watched.
• Make sure your baby feels no pain by giving pain medication.
• Make sure to look after your newborn’s medications.
• Manage the tubes (drains) that were placed in your baby’s chest during surgery.
• Take care of the chest wound (sternal incision) and any other incision sites.
• Make sure that your newborn is able to feed well by mouth.
• Will give you instructions to follow during your baby’s recovery.