Overview

An atrioventricular (AV) canal defect is a congenital (present at birth) heart condition. It describes a group of several heart issues, including a hole in the wall (septum) at the center of your heart and problems with the way your heart’s valves work.

Having a hole in the wall that separates the right and left sides of your heart is like a pipe with a leak. It allows oxygen-rich blood (normally from the left side of your heart) to mix with oxygen-poor blood (normally from the right side of your heart). These don’t mix in a properly functioning heart.

In most cases, the hole allows too much blood to flow into your lungs and not enough blood to flow to the rest of your body. If the hole is present for a long time, blood with less oxygen may flow in the opposite direction, with more of that blood going to your body than your lungs. This can result in lower oxygen levels and cyanosis (bluish coloring of your skin).

By making your heart work too hard to pump blood, an AV canal defect can lead to a range of health conditions like congestive heart failure.

AV canal defect can be fatal in children who don’t get treatment. Without an operation, children with this condition may only live two or three years. But the good news is that the surgery to fix this has a high success rate. Providers can diagnose an atrioventricular canal defect in a baby before it can cause fatal complications.

About 1 in 1,700 babies are born with this defect each year in the U.S. It makes up between 3% and 5% of all congenital heart defects.

Other names for AV canal defect include:

  • Atrioventricular septal defect.
  • AVSD.
  • Atrioventricular canal defect.
  • Endocardial cushion defect.

Types of atrioventricular canal defect

An atrioventricular canal defect can take various forms, with differences in hole size and number of valves.

Complete atrioventricular canal defect

The hole is big enough to make an opening between all four chambers of your heart. Instead of the typical two valves between the upper and lower chambers, there’s just one valve. Its flap might not open and shut correctly.

Partial atrioventricular canal defect

The hole is only between the wall of the two upper chambers (most typical) or the two lower chambers, not all four. There are two valves between the upper and lower chambers. But one of them (usually the mitral valve between the upper left atrium and lower left ventricle) doesn’t work correctly.

Transitional atrioventricular canal defect

There’s a hole between the two upper chambers and a smaller hole between the two lower chambers. The two AV valves are separate.

Unbalanced complete atrioventricular canal defect

The single (shared) AV valve is closer to one ventricle (lower chamber) than the other. The ventricle that gets more blood flow gets bigger than the other one. Providers call the smaller ventricle “hypoplastic.”

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Symptoms

When to see a doctor

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Complications

Blood clots are a dangerous complication of atrial fibrillation (AFib). Blood clots can lead to stroke.
The risk of stroke from AFib increases as you grow older. Other health conditions also may increase the risk of a stroke due to AFib. These conditions include:
  • High blood pressure.
  • Diabetes.
  • Heart failure.
  • Some types of heart valve disease.
Blood thinners are commonly prescribed to prevent blood clots and strokes in people with atrial fibrillation.

Prevention

Healthy lifestyle choices can reduce the risk of heart disease and may prevent atrial fibrillation (AFib). Here are some basic heart-healthy tips:
  • Control high blood pressure, high cholesterol and diabetes.
  • Don't smoke or use tobacco.
  • Eat a diet that's low in salt and saturated fat.
  • Exercise at least 30 minutes a day on most days of the week unless your health care team says not to.
  • Get good sleep. Adults should aim for 7 to 9 hours daily.
  • Maintain a healthy weight.
  • Reduce and manage stress.


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