How Do You Treat Atrioventricular Canal Defect?

Complications

• It is not uncommon for the valves to leak slightly. If the situation worsens, they may need to be repaired or replaced with artificial valves.
• If an artificial valve is implanted, the child will need to take anticoagulant medication to prevent blood clots from forming.
• During surgery, the electrical system of the heart can be damaged. This can result in a slow heartbeat, called heart block. This may necessitate the placement of a pacemaker in the child.
• Some patients develop sub-aortic stenosis, a blockage beneath the aortic valve that may require open heart surgery to repair.

Prognosis

• Failure to repair the defect may result in irreversible damage to the lungs' blood vessels (pulmonary vascular obstructive disease).
• As the blood vessels deteriorate, less blood flows to the lungs, resulting in bluish discoloration of the skin.
• An untreated defect can eventually lead to death. The goal of surgical repair within the first year of life is to avoid this outcome.
• The prognosis is favorable following a successful surgery. Children who undergo the operation are more likely to live a normal, healthy life. Care, however, is ongoing and necessitates yearly check-ups to track progress and monitor potential complications, such as a leaky heart valve.
• Children will be required to take preventative antibiotics before any dental or surgical procedures. These preventative antibiotics help reduce the risk of complications, such as endocarditis (a bacterial infection that occurs within the lining of the heart and can be fatal).
• Adults may not require any additional surgery. Women who are planning to have children should have an atrioventricular canal defect evaluated by a cardiologist.
• In most cases, more than one surgery is required.
• The mortality rate varies with the defect and age:
  • Partial defect: 10-year survival is more than 98%.
  • Complete defect: Operative mortality is 3%-5% and 10-year survival is more than 90%.

Atrioventricular canal defect can involve only the two upper chambers (atria) of the heart (partial) or all four chambers (complete). In either type, extra blood circulates in the lungs.

Complete atrioventricular canal defect

Typically, signs and symptoms appear within the first few weeks of life. These signs and symptoms are generally similar to those associated with heart failure:

• Difficulty breathing or rapid breathing
• Wheezing
• Fatigue
• Lack of appetite
• Poor weight gain
• Pale skin color
• Bluish discoloration of the lips and skin
• Excessive sweating
• Irregular or rapid heartbeat
• Swelling in the legs, ankles and feet (edema)

Partial atrioventricular canal defect

Signs and symptoms may not appear until early adulthood and may be related to complications caused by the defect:

• Abnormal heartbeat (arrhythmia)
• Shortness of breath
• High blood pressure in the lungs (pulmonary hypertension)
• Heart valve problems
• Heart failure

When to seek medical help

Contact a doctor immediately if the following signs are observed in children:

• Fast breathing
• Poor feeding
• Slow growth
• Bluish skin color
• Irritability
• Lowered alertness
• Wheezing
• Swelling of the legs, ankles, and feet
• Excessive sweating
• Fast heartbeat
• Sudden weight gain

It is unclear why atrioventricular canal defects occur. Factors that may increase the risk of developing this condition include:

• History of congenital heart defects in the family
• Down syndrome and other chromosomal disorders
• Previous pregnancy with fetal heart defects or miscarriage
• Health conditions in the mother, such as:
  • Viral infection
  • Poorly controlled diabetes
  • Alcohol consumption
  • Certain medications

During pregnancy, an ultrasound may be used to diagnose the child. When listening to the child’s heart with a stethoscope, the doctor may hear an abnormal sound (heart murmur) that leads them to suspect a heart defect. The doctor will be able to make a diagnosis based on the location and type of the murmur. Tests that may be ordered include:

• Chest X-ray: A chest X-ray may reveal heart and lung changes caused by an atrioventricular canal defect.
• Electrocardiogram: The electrical activity of the heart is recorded during this test. It detects abnormal heart rhythms (arrhythmias) and heart muscle stress. These problems could be caused by an atrioventricular canal defect.
• Echocardiogram: This test employs sound waves to create a moving image of the heart and its valves. The pattern of blood flow through the septal openings can be visualized using an echocardiogram. It can reveal the size of the openings and the amount of blood flowing through them. An echocardiogram is used to diagnose the majority of atrioventricular canal defects.
• Cardiac catheterization: Cardiac catheterization provides extremely detailed information about the structures within the heart. A small, thin, flexible tube (catheter) is inserted into a blood vessel in the child's groin during this test. The doctor then directs it to the child's heart. To see the child's heart more clearly, a contrast dye may be infected. Under sedation, the child's blood pressure and oxygen levels will be monitored.
• Genetic counseling: Because genetic factors may cause an atrioventricular canal defect, a genetic counselor and a cardiologist may help if there is a family history of heart defects or if a person already has had a child with a congenital heart defect. One of the most effective preventive strategies against congenital heart defects has been rubella vaccination.