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Atrioventricular (AV) canal defect, also known as AV canal defect, refers to a large hole in the center of the heart that prevents the separation of all four heart chambers and requires surgical repair.

The heart’s left side should pump blood out into the rest of the body, and the right side should send blood only to the lungs. In patients with AV canal defects, blood flows from the left to the right chambers and then into the lungs through the arteries. As a result, the heart and lungs work harder because of the extra blood going into the lungs, and lung congestion can occur because of high pressure in those blood vessels.

This can lead to pulmonary hypertension into adulthood, as well as permanent lung damage. There also may be regurgitation (leaking) that can create heart failure symptoms.

Causes & risk factors

AV canal defects often are associated with trisomy 21 (Down syndrome), though most patients may have normal chromosomes. Others may have additional heart problems. Patients with AV canal defect may have a hole between the heart’s upper chambers (atrial septal defect) without a hole separating the lower chambers (ventricular septal defect) and vice versa. They may also have abnormalities of the tricuspid and mitral valves.

Once the defect has been repaired and healed, patients can usually return to normal activity without any particular precautions. Pulmonary hypertension patients and those with unrepaired defects who have heart failure usually have decreased ability for exercise and a shortened life span. 


Symptoms of AV canal defect in infants and children include:

  • Poor weight gain
  • Sweating during feeding
  • Tachypnea (rapid and shallow breathing)
  • Congestive heart failure symptoms

Though AV canal defects are diagnosed prenatally and in infancy, adults with unrepaired AV canal defects can experience symptoms, which include: 

  • Heart murmurs due to valve leaks and blood flow across the hole
  • Shortness of breath
  • Decreased exercise capacity
  • Palpitations

Diagnosis & testing

A cardiologist can evaluate your child for AV canal defect with noninvasive tests, such as:

  • Electrocardiogram
  • Echocardiograms
  • Holter monitor
  • Exercise stress tests
  • Cardiac MRI

Sometimes, invasive tests are needed as well, including heart catheterization to determine whether the patient is a surgical candidate. 

Treatment options

Prior to surgical intervention, medical therapy may be employed to treat congestive heart failure symptoms and encourage infant weight gain. Patients with valve regurgitation will likely need heart failure medications to help their hearts pump better and/or lower their blood pressure. Patients with pulmonary hypertension may also require medications. 

Surgery for AV canal defect is usually performed in infancy approximately 3-6 months of age. Lifelong surveillance is required to assess the valve, the possible development of left ventricular outflow obstruction, and the development of rhythm (electrical) problems.

How well the repaired valves perform must be monitored long term. Some valves may need to be replaced with a mechanical one and occasionally can be repaired. 

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