Diagnosis

Atrioventricular canal defect might be detected in a baby before birth using ultrasound and special heart imaging.

After birth, signs and symptoms of complete atrioventricular canal defect are usually noticeable within the first few weeks. When listening to your baby's heart, your doctor might hear an abnormal whooshing sound (heart murmur).

If your baby is having signs and symptoms of atrioventricular canal defect, your doctor might recommend the following tests:

  • Electrocardiogram (ECG or EKG). Electrical signals are recorded as they travel through the heart. Your doctor can look for patterns that show abnormal heart function.
  • Echocardiogram. Sound waves are used to create pictures of the heart in motion. An echocardiogram can reveal a hole in the heart, abnormal heart valves and abnormal blood flow through the heart.
  • Chest X-ray. A chest X-ray can show an enlarged heart.
  • Cardiac catheterization. A thin, flexible tube (catheter) is inserted into a blood vessel in the groin and up to the heart. A dye injected through the catheter makes the heart structures show up more clearly on X-rays. The catheter also allows the doctor to measure pressure in the chambers of the heart and in the blood vessels.

Treatment

Surgery is needed to repair a complete or partial atrioventricular canal defect. More than one surgery may be needed. Surgery to correct atrioventricular canal defect involves using one or two patches to close the hole in the heart wall. The patches stay in the heart permanently, becoming part of the heart's wall as the heart's lining grows over them.

Other surgeries depend on whether you have a partial or complete defect and what other heart problems you may have. For a partial atrioventricular canal defect, surgery to repair the mitral valve is needed so that the valve will close tightly. If repair isn't possible, the valve might need to be replaced.

For a complete atrioventricular canal defect, surgeons separate the abnormal large single valve between the upper and lower heart chambers into two valves. If separating the single valve isn't possible, heart valve replacement of both the tricuspid valve and the mitral valve might be needed.

After surgery

If the heart defect is repaired successfully, you or your child will likely have no activity restrictions.

You or your child will need lifelong follow-up care with a cardiologist trained in congenital heart disease. Your cardiologist will likely recommend a follow-up exam once a year or more frequently if problems, such as a leaky heart valve, remain.

Adults whose congenital heart defects were treated in childhood may need care from a cardiologist trained in adult congenital heart disease (adult congenital cardiologist) throughout life. Special attention and care may be needed around the time of any future surgical procedures, even those that do not involve the heart.

You or your child might also need to take preventive antibiotics before certain dental and other surgical procedures if either of you:

  • Have remaining heart defects after surgery
  • Received an artificial heart valve
  • Received artificial (prosthetic) material during heart repair
  • Will receive a heart valve prosthetic in the next six months

The antibiotics are used to prevent inflammation and infection of the lining of the heart (endocarditis).

Many people who have corrective surgery for atrioventricular canal defect don't need additional surgery. However, some complications, such as heart valve leaks, may require treatment.

Coping and support

Having a congenital heart defect or caring for a child with a congenital heart defect can be challenging. To help make it easier:

  • Seek support. Ask for help from family members and friends. Talk with your or your child's cardiologist about support groups and other types of assistance that are available near you.
  • Record your or your child's health history. Write down medications, surgery and other procedures and the dates they were performed. Include the medical report from the surgeon, and any other important information about your or your child's care. This information will be useful for doctors who are unfamiliar with you or your child. It will also help your child transition from pediatric to adult doctors.
  • Talk about your concerns for your child. Many children with atrioventricular canal defect will have no limitations. But talk with the cardiologist about activities that are safe for your child. If some are off-limits, encourage your child in other pursuits rather than focusing on what he or she can't do.

Although every circumstance is different, remember that many children with congenital heart defects grow up to lead healthy lives.

Prevention

Atrioventricular canal defect generally can't be prevented.

Some heart defects are passed down in families (inherited). If you have a family history of heart defects or if you already have a child with a congenital heart defect, talk with a genetic counselor and a cardiologist before getting pregnant again.

Preparing for your appointment

You or your child might be referred to a doctor trained in heart conditions (cardiologist).

What you can do

  • Write down any symptoms you or your child has, including any that may seem unrelated to the reason why you scheduled the appointment.
  • Make a list of all your medications, vitamins and supplements.
  • Write down important medical information, including other health conditions you or your child may have.
  • Write down key personal information, including any recent changes or stress in your life.
  • Write down questions to ask your doctor.
  • Find out if your family has a history of heart disease.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may leave time to go over points you want to spend more time on. You may be asked questions such as:

  • When did you first notice symptoms? Do they come and go or do you always have them?
  • Does anything seem to improve or worsen these symptoms?
  • Do you have a family history of congenital heart disease?
  • Did you have diabetes or a viral infection, such as measles, during your pregnancy?
  • Did you take any medications during pregnancy?
  • Did you use tobacco or smoke during pregnancy?

Questions to ask your doctor

Preparing a list of questions can help you make the most of your time with your doctor. For atrioventricular canal defect, some basic questions to ask your doctor include:

  • What's the most likely cause of my symptoms or my baby's symptoms?
  • What tests are needed? Is there any special preparation for them?
  • What kind of treatment do you recommend?
  • How can we manage other health problems together with atrioventricular canal defect?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions during your appointment.

July 23, 2021
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