About Atrioventricular Septal Defect

Atrioventricular Canal Defect Animation

An animation depicting a heart with atrioventricular canal defect.

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Complete Common Atrioventricular Canal Defect, also known as Atrioventricular Septal Defect, is a lack of separation of the atria and the ventricles into separate right and left chambers as well as a lack of separation of the mitral and tricuspid valves. The lack of separation of these two valves results in a single atrioventricular valve.

In patients with atrioventricular canal defect, the single atrioventricular may not close properly. Therefore, the heart must pump an excessive amount of blood and this may result in an enlargement of the heart.

Surgical repair for atrioventricular canal defect is required within the first six months of life. The surgery involves sewing patches over the ASD and VSD and carefully separating the single atrioventricular valve into two valves.


How Does Atrioventricular Canal Defect Differ From
Normal Cardiac Anatomy?

If your child has atrioventricular canal defect the structure of his or her heart is different from normal cardiac anatomy.

 
Learn More About Normal Cardiac Anatomy

Heart With Normal Cardiac Anatomy

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When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.

The heart is composed of four chambers. The two upper chambers, known as atria, collect blood as it flows back to the heart. The two lower chambers, known as ventricles, pump blood with each heartbeat to the two main arteries (the pulmonary artery and the aorta). The septum is the wall that divides the heart into right and left sides. The atrial septum separates the right and left atria; likewise, the ventricular septum separates the two ventricles.

There are four valves that control the flow of blood through the heart. These flap-like structures allow blood to flow in only one direction. The tricuspid and mitral valves, also known as the atrioventricular valves, separate the upper and lower chambers of the heart. The aortic and pulmonary valves, also known as the arterial valves, separate the ventricles from the main arteries. Oxygen-depleted blood returns from the body and drains into the right atrium via the superior and inferior vena cavas. The blood in the right atrium then passes through the tricuspid valve and enters the right ventricle.

Next, the blood passes through the pulmonary valve, enters the pulmonary artery, and travels to the lungs where it is replenished with oxygen. The oxygen-rich blood returns to the heart via the pulmonary veins, draining into the left atrium. The blood in the left atrium passes through the bicuspid, or mitral, valve and enters the left ventricle.

Finally, the oxygen-rich blood flows through the aortic valve into the aorta and out to the rest of the body.

When Your Child Needs a Heart Transplant

What Is a Heart Transplant?

A heart transplant is a surgery in which doctors remove a person's sick heart and replace it with a healthy donor heart.

Transplants are done when a child's heart does not work well and he or she won't survive without a new one. Doctors sometimes call this heart failure, or end-stage pediatric heart disease. They usually first try to treat heart failure with medicine, surgery, or other procedures. If those don't work, a child might need a heart transplant.

Transplanted hearts come from organ donors who have agreed (or their guardians have agreed) to donate their organs when they die. They choose to donate the organs because they want to help someone else who is sick.

Many kids who have heart transplants go on to live normal, healthy lives after they recover from surgery. They will take medicines for the rest of their lives to prevent the body from rejecting the heart. Rejecting means that the body's immune cells attack the new heart because they sense that it's foreign.

What Happens Before a Heart Transplant?

If your child needs a heart transplant, your doctor will refer you to a transplant center. There, you'll meet the members of the transplant team, which usually includes:

  • surgeons
  • cardiologists (heart doctors)
  • a transplant coordinator
  • nurses
  • dietitians
  • psychologists
  • social workers

The health care team will check to make sure that your child is healthy enough to have surgery and take the medicines needed after it. The team will do tests such as:

  • blood tests: to determine blood type
  • an echocardiogram: a test in which sound waves make an image of the heart
  • an electrocardiogram (also known as an ECG or EKG): a test that records the electrical activity of the heart and can help find arrhythmias and heart muscle damage
  • cardiac catheterization: a procedure in which a thin, plastic tube called a catheter is put into a blood vessel and then threaded through to the heart so doctors can view the heart and its chambers

The doctor also might do a biopsy, removing a tiny piece of tissue from the heart to examine under a microscope.

The transplant evaluation lets the team learn as much about your child as possible. But it's also a time for you and your child to learn about what will happen before, during, and after the transplant. The transplant team is there to provide information and support. Be sure to ask if you don't understand something.

If the transplant team decides your child is a good candidate, the next step is to find a heart. Your child's name will go on an organ waiting list. This list has the names of everyone who is waiting for a heart or other organs.

Your child might have to wait to find a heart that is a good match. The need for new hearts is far greater than the number donated, so this can take a long time.

You'll stay in close touch with the doctors and the rest of the health care team. Make sure they know how to reach you at all times. When a heart is available, you'll need to move quickly. Keep a bag packed and be ready to go to the transplant hospital at a moment's notice.

While you wait for a transplant, keep your child as healthy as possible. That way, he or she will be ready for transplant surgery when the time comes. Help your child:

  • eat healthy foods
  • take all medicines as directed
  • keep all medical appointments

Tell your doctor and the transplant center right away if is any change in your child's health.

What Happens During Heart Transplant Surgery?

When you get to the hospital, the transplant team will prepare your child for surgery. They may run a few tests to be sure that the new heart is a good match. Then, your child goes to an operating room.

In the operating room, your child will get anesthesia to sleep through the operation. The surgeon makes an incision (cut) in the chest and removes the sick heart. The new heart is placed in the chest, and the surgeon connects it by sewing its blood vessels to the blood vessels in your child's body. So that doctors can check heart function, the incision might not be closed right away.

Most heart transplant surgeries last between 4 and 6 hours. Someone on the transplant team will keep you informed about how the surgery is going while you wait.

What Happens After Heart Transplant Surgery?

After heart transplant surgery, your child be in the cardiac intensive care unit (CICU). You can visit your child there for brief periods. Your child will get medicine for pain. He or she will stay asleep on the ventilator with a breathing tube in place until the doctors know that the new heart is working well. This may take a few days.

How long your child will be in the CICU depends on his or her condition. Generally, the stay is 7 to 10 days. When ready, your child will be transferred to the general cardiac ward. The transplant team will continue to care for and closely watch your child.

Most children stay in the hospital at least 3 to 4 weeks after surgery. During this time, kids and their families learn how to care for the new heart. Be sure you understand the doctors' instructions because your child will need to follow them carefully.

In the weeks after your child goes home, you'll return to the hospital many times so that the doctors can make sure that everything is going well.

What Problems Can Happen?

One of the most common problems after transplant surgery is rejection. Rejection happens because the body doesn't recognize the new heart and doesn't know that it is helpful. So the immune system tries to attack it.

Medicines (called immunosuppressants, or anti-rejection medicines) help to control this reaction. In a sense, they trick the body into accepting the new heart. Taking them can make your child more likely to get infections, especially in the days right after surgery. So keep your child away from sick people, and have everyone at home wash their hands well and often.

The risk of rejection is greatest in the first few weeks after transplant surgery. But the body never completely accepts the new heart. So anti-rejection medicines are taken for life.

Usually, the amount of immunosuppressants taken is reduced as the body gets used to the new heart. Rarely, the body refuses to accept the new organ and another transplant is needed.

Looking Ahead

Many kids who have heart transplants live normal, healthy lives after they recover from surgery. Some feel better than they ever have before.

It's very important for you and your child to do everything possible to keep the new heart healthy. Make sure that your child takes all medicines as directed, and encourage him or her to get plenty of rest, exercise regularly, and eat well.

Also, watch your child for signs of infection or rejection, including:

  • fever
  • tiredness
  • trouble breathing
  • vomiting
  • weight gain
  • poor appetite

Call your doctor right away if these or any other problems happen.

Your child will have regular checkups so doctors can watch for problems. At first, these visits might happen weekly. You'll go less often over time. Eventually, checkups might be needed only once or twice a year.

How Can I Help My Child?

Having a serious condition can be hard for kids. Surgery and immunosuppressant therapy can add to the stress. Talk to your child about these changes and how you will work them into your routine. Make sure to find time to do fun things together with family and friends.

For teens, immunosuppressant therapy can be a challenge. These medicines can cause:

  • more acne
  • weight gain

These side effects are a major reason why teens are at risk for not taking their medicines after a transplant. This can be dangerous and even lead to rejection of the new heart. So be sure to talk about the importance of taking all medicines as directed.

To help:

  • Be there for your child to talk to.
  • If your child needs more support, make an appointment with a therapist or counselor.
  • Find a support group. They're a great way for kids and teens to relieve stress and connect with others who are going through similar challenges. Online resources include Transplant Living.
  • Get support for yourself too. It can be a huge relief to talk about your feelings with other people who know what you're going through. Ask the transplant care team if they know of support groups for families.

Reviewed by: Easton Kernich, MSN, NP-C
Date reviewed: November 19, 2018