Treatment for TAPVR
Specific treatment for TAPVR will be determined by your child's doctor based on:
Your child's age, overall health, and medical history
Extent of the disease
Your child's tolerance for specific medications, procedures, or therapies
Expectations for the course of the disease
Your opinion or preference
Your child may be admitted to the intensive care unit (ICU) or special care nursery once symptoms are noted. Initially, your child may be placed on oxygen, and possibly even on a ventilator, to assist his or her breathing. IV medications may be given to help the heart and lungs function more efficiently.
Medical management. Initial medical management may include oxygen, medications to increase blood pressure, and treatments with prostaglandins, a medication used to keep the ductus arteriosus open (although this medication may be contraindicated in certain forms of the disease).
Interventional cardiac catheterization. A cardiac catheterization procedure can be used as a diagnostic procedure, as well as initial treatment procedure for some heart defects. A cardiac catheterization procedure will usually be performed to evaluate the defect(s) and the amount of blood that is mixing. As part of the cardiac catheterization, a procedure called balloon atrial septostomy may be performed to improve mixing of oxygen-rich (red) and oxygen-poor (blue) blood:
A special catheter with a balloon in the tip is used to create an opening in the atrial septum (wall between the left and right atria).
The catheter is guided through the foramen ovale—a small opening present in the atrial septum that closes shortly after birth—and into the left atrium.
The balloon is inflated.
The catheter is quickly pulled back through the hole, into the right atrium, enlarging the hole, allowing blood to mix between the atria.
An IV medication called prostaglandin E1 may be given to keep the ductus arteriosus from closing.
Surgical repair. The operation is performed under general anesthesia. The four pulmonary veins are reconnected to the left atrium, and any associated heart defects such as atrial septal defect, ventricular septal defect, patent foramen ovale, and/or patent ductus arteriosus are surgically closed.
Postoperative care for your child
After surgery, infants will return to the intensive care unit (ICU) for a few days to be closely monitored during recovery.
While your child is in the ICU, special equipment will be used to help him or her recover, and may include the following:
Ventilator. A machine that helps your child breathe while he or she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he or she is too sleepy to breathe effectively on his or her own. After a TAPVR repair, children will benefit from remaining on the ventilator overnight or even longer so they can rest.
Intravenous (IV) catheters. Small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medicines that help your child recover from the operation.
Arterial line. A specialized IV placed in the wrist or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the ICU.
Nasogastric (NG) tube. A small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.
Urinary catheter. A small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and, therefore, the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys to remove excess fluid from the body.
Chest tube. A drainage tube may be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.
Heart monitor. A machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values.
Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.
Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.
After discharged from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.
Infants who spent a lot of time on a ventilator, or who were fairly ill while in the ICU, may have trouble feeding initially. These babies may have an oral aversion; they might equate something placed in the mouth, such as a pacifier or bottle, with a less pleasant sensation such as being on the ventilator. Some infants just are tired, and need to build their strength up before they will be able to learn to bottle-feed. Strategies used to help infants with nutrition include the following:
High-calorie formula or breast milk. Special nutritional supplements may be added to formula or pumped breast milk that increase the number of calories in each ounce, thereby allowing your baby to drink less and still consume enough calories to grow.
Supplemental tube feedings. Feedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, that can either supplement or take the place of bottle-feedings. Infants who can drink part of their bottle, but not all, may be fed the remainder through the feeding tube. Infants who are too tired to bottle-feed at all may receive their formula or breast milk through the feeding tube alone.
Caring for your child at home following a TAPVR surgical repair
Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable at home. Your child's doctor will discuss pain control before your child is discharged from the hospital.
If any special treatments are to be given at home, the nursing staff will ensure that you are able to provide them, or a home health agency may assist you.
You may receive additional instructions from your child's doctors and the hospital staff.
Long-term outlook after TAPVR surgical repair
Many infants who have had TAPVR surgical repair will grow and develop normally. However, after TAPVR repair, your infant will need to be followed periodically by a pediatric cardiologist who will make assessments to check for any heart-related problems.
As some children grow, the area where the pulmonary arteries were reconnected to the left atrium may become narrowed, preventing blood from moving from the lungs to the left atrium. Treatment may include:
Placement of a wire mesh device called a stent into the narrowed vein(s) to open it, done during a cardiac catheterization procedure
Surgical enlargement of the narrowed pulmonary vein connection(s)
Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individual’s lifespan.
Consult your child's doctor regarding the specific outlook for your child.