Atrioventricular canal defect is a combination of heart problems causing a defect in the center of the heart. The condition happens when there's a hole between the heart's chambers and problems with the valves that regulate blood flow in the heart.
Sometimes referred to as endocardial cushion defect or atrioventricular septal defect, atrioventricular canal defect is present at birth (congenital). The situation is often related with Down syndrome.
Atrioventricular canal defect allows more blood to go to the lungs. The extra blood pressures the heart to overwork, making the heart muscle to enlarge.
If not treated, atrioventricular canal defect can lead to heart failure and high blood pressure in the lungs. Doctors usually recommend surgery during the first year of life to cover the hole in the heart and to reconstruct the valves.
Atrioventricular canal defect is divided into partial and complete. In any of the two, extra blood circulates in the lungs.
Complete atrioventricular canal defect
Signs and symptoms generally develop in the first several weeks of birth. These include:
Difficulty breathing or rapid breathing
Poor weight gain
Irregular or rapid heartbeat
Lack of appetite
Pale skin color
Bluish discoloration of the lips and skin
Signs may not be noticed until early adulthood and might be related to problems that develop as a result of the defect. These signs and symptoms might include:
Abnormal heartbeat (arrhythmia)
Shortness of breath
High blood pull in the lungs (pulmonary hypertension)
Heart valve issues
The right side of your heart moves blood into vessels that go to the lungs. There, oxygen enriches the blood. The oxygen-rich blood flows back to your heart's left side and is pumped into a large vessel (aorta) that circulates blood to every part of the body of your body.
Valves manage the flow of blood in and out of the chambers of your heart. These valves give way to allow blood to move to the other chamber or to one of the arteries, and close to keep blood from flowing backward.
In partial atrioventricular canal defect:
There's a hole in the wall (septum) that divides the higher chambers (atria) of the heart.
Sometimes the valve between the upper and lower left chambers (mitral valve) also has a defect that makes it to leak (mitral valve regurgitation).
In complete atrioventricular canal defect:
There's a large hole in the center of the heart the place the walls between the atria and the lower chambers (ventricles) meet.
Instead of different valves on the right and left, there's one large valve between the upper and lower chambers.
The abnormal valve drips blood into the ventricles.
The heart is pressured to work harder and enlarges.
Factors that may enlarge a baby's risk of developing atrioventricular canal defect prior to birth include:
Smoking when pregnant
German measles (rubella) or every other viral illness during a mother's early pregnancy
Alcohol intake during pregnancy
Poorly controlled diabetes throughout pregnancy
Certain medications taken when pregnant — discuss with your doctor before taking any drugs while you're pregnant or trying to become pregnant
Someone whose parent had a congenital heart defect
Complications of atrioventricular canal defect may include:
Enlargement of the heart. Increased blood goes with the flow through the heart pressures it to work harder than normal, making it to enlarge.
Pulmonary hypertension. When there is a hole (defect) that allows mixing of oxygenated (red) and deoxygenated (blue) blood, the amount of blood that flows to the lungs is increased. This results in pressure buildup in the lungs, resulting to high blood pressure in the lungs.
Respiratory tract infections. Atrioventricular canal defect can cause intermittent bouts of lung infections.
Heart failure.If not treated, atrioventricular canal defect generally results in the heart's inability to pump enough blood to meet the body's requirements.
Treatment highly improves the outlook for children with atrioventricular canal defect. Nevertheless, some children who have corrective surgery may be at risk later in life of:
Narrowing of the heart valves
Abnormal heart rhythm
Leaky heart valves
Breathing difficulties associated with lung damage
Rapid, fluttering heartbeat
Shortness of breath
Atrioventricular canal defect typically can't be prevented.
Heredity may additionally play a role in some heart defects. If you have a family history of heart defects or if you already have a child with a congenital heart defect, discuss with a genetic counselor and a cardiologist before getting pregnant again.
Immunization with rubella vaccine has been one of the most effective preventive methods against congenital heart defects.
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