Diagnosis

Atrioventricular canal defect might be detected before birth through 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) caused by turbulent blood flow.

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

  • Electrocardiogram (ECG or EKG). Electrical signals are recorded as they travel through the heart. Your doctor can look for patterns that indicate abnormal heart function.
  • Echocardiogram. Sound waves produce live-action images of the heart. Echocardiogram can reveal a hole in the heart and abnormal heart valves, as well as abnormal blood flow through the heart.
  • Chest X-ray. The image can show evidence of 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 visible on X-ray pictures. 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 complete and partial atrioventricular canal defects. The procedure involves closing the hole in the wall (septum) between the heart chambers with one or two patches. The patches stay in the heart permanently, becoming part of the septum as the heart's lining grows over them.

For a partial atrioventricular canal defect, surgery also involves repair of the mitral valve, so it will close tightly. If repair isn't possible, the valve might need to be replaced.

For a complete atrioventricular canal defect, surgery also includes separation of the large single valve that separates the upper and lower chambers of the heart into two valves, on both the left and right sides of the repaired septum. If separating the single valve isn't possible, heart valve replacement of both the tricuspid and mitral valves might be needed.

After surgery

If the heart defect is repaired successfully, your child will likely lead a normal life, often with no activity restrictions.

However, 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 as children 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 procedures, such as an operation which does not involve the heart.

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

  • Has remaining heart defects after surgery
  • Received an artificial heart valve
  • Received artificial (prosthetic) material during heart repair

The antibiotics are used to prevent a bacterial 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.

Pregnancy

Women who had an atrioventricular canal defect that was surgically corrected before any permanent lung damage occurred can generally expect to have normal pregnancies. However, pregnancy isn't recommended if serious heart or lung damage occurred before surgery.

Evaluation by a cardiologist trained in congenital heart disease (adult congenital cardiologist) is recommended for women with repaired or unrepaired atrioventricular canal defect before they attempt pregnancy.

Coping and support

Having a congenital heart defect or caring for a child with a congenital heart defect can be scary and 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 your or your child's diagnosis, medications, surgery and other procedures and the dates they were performed. Include the operative 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 baby. It will also help your child transition from pediatric to adult doctors.
  • Talk about your concerns for your child. Many children 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.

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 symptoms you or your child experiences, 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 key medical information, including other conditions.
  • Write down key personal information, including any recent changes or stressors 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? Are they continuous or occasional?
  • Does anything seem to improve or worsen these symptoms?
  • Is there a family history of congenital heart disease?
  • Did you have diabetes or a viral infection, such as measles, during your pregnancy?
  • Were any medications taken during pregnancy?
  • Was there tobacco or alcohol use during pregnancy?

Questions to ask your doctor

Preparing a list of questions can help you make the most of your time with your doctor, and ensure that you cover all of the points that are important to you. 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.

Oct. 10, 2017
References
  1. Fleishman C, et al. Clinical manifestations, pathophysiology, and diagnosis of atrioventricular (AV) canal defects. https://www.uptodate.com/contents/search. Accessed May 25, 2017.
  2. Fleishman C, et al. Management and outcome of atrioventricular (AV) canal defects. https://www.uptodate.com/contents/search. Accessed May 25, 2017.
  3. Karamlou T, et al. Congenital heart disease. In: Schwartz's Principles of Surgery. 10th ed. New York, N.Y.: The McGraw-Hill Companies; 2015. http://www.accessmedicine.mhmedical.com. Accessed May 25, 2017.
  4. What are holes in the heart? National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health/health-topics/topics/holes. Accessed May 25, 2017.
  5. Sutton M, et al. Clinical manifestations and diagnosis of atrial septal defects in adults. https://www.uptodate.com/contents/search. Accessed May 25, 2017.
  6. Lin J, et al. Congenital heart disease in adolescents and adults. In: Hurst's The Heart. 14th ed. New York, N.Y.: McGraw-Hill Education; 2017.
  7. The impact of congenital heart defects. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/TheImpactofCongenitalHeartDefects/The-Impact-of-Congenital-Heart-Defects_UCM_001218_Article.jsp. American Heart Association. Accessed June 9, 2017.
  8. Ropper A, et al. Developmental diseases of the nervous system. In: Adams and Victors Principles of Neurology. 10th ed. New York, N.Y.: McGraw-Hill Education; 2014.

Atrioventricular canal defect