Treatment for atrioventricular canal
Specific treatment for atrioventricular canal will be determined by your child's physician 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
AV canal is treated by surgical repair of the defects. However, medical support (i.e., medications) may be necessary until the operation is performed. Treatment may include:
Medical management. Many children will eventually need to take medications to help the heart and lungs work better, due to strain from the extra blood passing through the septal defects. Medications that may be prescribed include the following:
Digoxin. Helps strengthen the heart muscle, enabling it to pump more efficiently.
Diuretics. The body's water balance can be affected when the heart is not working as well as it could. These medications help the kidneys remove excess fluid from the body.
ACE (angiotensin-converting enzyme) inhibitors. Dilates the blood vessels, making it easier for the heart to pump blood forward into the body.
Adequate nutrition. Infants may become tired when feeding, and may not be able to eat enough to gain weight. Options that can be used to ensure your baby will have adequate nutrition include:
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 properly.
Supplemental tube feedings. Feedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, 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 may receive their formula or breast milk through the feeding tube alone.
Infection control. Children with certain heart defects are at risk for developing an infection of the valves of the heart known as bacterial endocarditis. It is important that you inform all medical personnel that your child has a an atrioventricular canal defect so they may determine if the antibiotics are necessary before any major procedure.
Surgical repair. The goal is to repair the septal openings and repair the valves before the lungs become damaged from too much blood flow and pressure. Your child's cardiologist will recommend when the repair should be performed based on results from the echocardiogram and/or cardiac catheterization.
The operative methods used to repair atrioventricular canal have improved greatly in the past decade, and the operation has a high likelihood of success. Most children undergo surgery by the age of 6 months. Children with Down syndrome may develop lung problems earlier than other children, and may need to have surgical repair at an earlier age.
The operation is performed under general anesthesia.
At surgery, the ventricular septal defect is often closed with a synthetic patch made from a polyester-like material known as Dacron. The atrial septal defect is often closed with a pericardial patch made from the membrane that covers the outside surface of the heart. The valve repair technique consists of converting the abnormal three-leaflet mitral valve into a two-leaflet mitral valve. This is accomplished by suturing the cleft (the cut in the valve leaflets) to recreate a two-leaflet (two-cusp) mitral valve.
Postoperative care for your child
Children will spend time in the intensive care unit (ICU) after an AV canal repair. During the first several hours after surgery, your child will be very drowsy from the anesthesia that was used during the operation, and from medications given to relax him or her and to help with pain. To help your child rest, these medications might be continued overnight or longer. As time goes by, your child will become more alert.
While your child is in the ICU, special equipment will be used to help him or her recover, and may include the following:
Ventilator. 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 AV canal repair, children will benefit from remaining on the ventilator overnight or even longer so they can rest. Children with Down syndrome may remain on the ventilator a bit longer than children without Down syndrome.
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 the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys remove excess fluids 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 of which relieve pain, and some of which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.
After discharge 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 at home, and these will be explained to you. The staff will give you written instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.
Caring for your child at home following AV canal repair
Most infants and older children feel comfortable when they go home. Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child's physician will discuss pain control before your child is discharged from the hospital.
Often, infants who fed poorly prior to surgery have more energy after the recuperation period, and begin to eat better and gain weight faster. However, high-calorie formulas may be needed for several weeks or months after surgery to help your child catch up growth-wise. Tube feedings may also be helpful until your child is able to feed better.
After surgery, older children usually have a fair tolerance for activity. Your child may become tired easily, and sleep more right after surgery, but, within a few weeks, your child may be fully recovered.
You may receive additional instructions from your child's physicians and the hospital staff.
Long-term outlook after AV canal surgical repair
Many children who have had an AV canal defect repair will live healthy lives. Activity levels, appetite, and growth will eventually return to normal in most children. Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis for a specific time period after discharge from the hospital.
Some children will still have some degree of mitral or tricuspid valve abnormality after AV canal repair surgery. This may require another operation in the future to repair the leaky or blocked valve(s).
Children with Down syndrome will benefit from special programs that enhance their mental and physical development. Your child's physician can assist you in locating such programs in your community.
Consult your child's physician regarding the specific outlook for your child.