Atrioventricular canal defect
Definition :
Atrioventricular canal defect is a combination of several abnormalities in the heart present at birth (congenital abnormalities). This defect, which is sometimes called endocardial cushion defect or atrioventricular septal defect, occurs when there's a hole between the chambers of the heart and problems with the valves that regulate blood flow in the heart.
Atrioventricular canal defect allows extra blood to circulate to the lungs. Ensuing problems overwork the heart and cause it to enlarge.
Atrioventricular canal defect is often associated with Down syndrome. If left untreated, atrioventricular canal defect may cause heart failure and high blood pressure in the lungs. To fix this defect, doctors often recommend surgery during the first year of life to close the hole and reconstruct the valves.
Symptoms:
There are two common types of atrioventricular canal defect — partial and complete. The partial form involves only the two upper chambers of the heart. The complete form allows blood to travel freely among all four chambers of the heart. In either type, extra blood circulates in the lungs.
Complete atrioventricular canal defect
Signs and symptoms of complete atrioventricular canal defect usually develop in the first several weeks of life. They include:
Signs and symptoms of a partial atrioventricular canal defect may not appear until early adulthood. When they do become noticeable, signs and symptoms may be related to complications that develop as a result of the defect, and may include:
Contact your doctor if you or your child develops any of the following signs or symptoms. These could be indications of heart failure or another complication of atrioventricular canal defect:
Causes:
Atrioventricular canal defect occurs during fetal growth when your baby's heart is developing. While some factors, such as Down syndrome, may increase the risk of atrioventricular canal defect, in most cases the cause is unknown.
The normal-functioning heart
Your heart is divided into four chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout your body — your heart uses its left and right sides for different tasks. The right side moves blood into vessels that lead to your lungs. In your lungs, oxygen enriches your blood, which circulates to your heart's left side. The left side of your heart pumps blood into a large vessel called the aorta, which circulates blood to the rest of your body. Valves control the flow of blood into and out of the chambers of your heart. These valves open to allow blood to move to the next chamber or to one of the arteries, and they close to keep blood from flowing backward.
What happens in atrioventricular canal defect
In partial atrioventricular canal defect, a hole exists in the wall (septum) that separates the upper chambers (atria) of the heart. Also, the mitral valve between the upper and lower left chambers often has a defect called a cleft that causes it to leak (mitral valve regurgitation).
In complete atrioventricular canal defect, there's a large hole in the center of the heart where the walls between the upper chambers (atria) and lower chambers (ventricles) meet. Instead of two separate valves — one on the right (tricuspid) and one on the left (mitral) — one large common valve exists between the upper and lower chambers. And, this valve may not close tightly.
Oxygen-rich and oxygen-poor blood mix through the hole in the septum, and the abnormal valves leak blood into the heart's lower chambers (ventricles). These problems make the heart work harder, causing it to enlarge.
Complications:
Treating an atrioventricular canal defect helps your child avoid potential complications, such as:
Although treatment greatly improves the outlook for children with atrioventricular canal defect, some who have corrective surgery may still be at risk of the following associated conditions later in life:
Treatments and drugs:
Surgery is necessary to fix both complete and partial atrioventricular canal defects. During the procedure, the hole in the septum is closed using one or two patches. The patches remain in the heart permanently, becoming part of the septum as the heart's lining grows over it. The surgeon also
separates the one large heart valve between the upper and lower chambers into two separate valves.
For a partial atrioventricular canal defect, the surgery also involves repair of the mitral valve so it will close tightly. If repair isn't possible, the valve may need to be replaced instead.
If your baby has complete atrioventricular canal defect, the surgery also includes separation of the single valve into two valves, one on the left side and one on the right side of the repaired septum. If reconstruction of the single valve into two valves isn't possible, heart valve replacement may be necessary.
After surgery
If the atrioventricular canal defect has been successfully repaired with surgery, your child will likely lead a normal life, often with no activity restrictions.
However, your child will need lifelong follow-up care with a heart doctor (cardiologist) who specializes in congenital heart disease. The recommendation for follow-up is usually once a year, unless you have lingering problems, such as a leaky heart valve. In that case, follow-up will be more frequent.
Your child may also need to take preventive antibiotics before certain dental procedures and other surgical procedures if he or she is at risk of severe complications of endocarditis, a bacterial infection of the lining of the heart. Typically, this is when your child has some remaining defects after surgery, has received an artificial valve or has had repair with artificial (prosthetic) material.
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
When an atrioventricular canal defect has been surgically corrected before any permanent lung damage has occurred, women can generally expect to have normal pregnancies. Pregnancy is not recommended, however, if serious heart or lung damage occurred before surgery. An evaluation by a cardiologist is advised before women with repaired or unrepaired atrioventricular canal defect attempt pregnancy.
Definition :
Atrioventricular canal defect is a combination of several abnormalities in the heart present at birth (congenital abnormalities). This defect, which is sometimes called endocardial cushion defect or atrioventricular septal defect, occurs when there's a hole between the chambers of the heart and problems with the valves that regulate blood flow in the heart.
Atrioventricular canal defect allows extra blood to circulate to the lungs. Ensuing problems overwork the heart and cause it to enlarge.
Atrioventricular canal defect is often associated with Down syndrome. If left untreated, atrioventricular canal defect may cause heart failure and high blood pressure in the lungs. To fix this defect, doctors often recommend surgery during the first year of life to close the hole and reconstruct the valves.
Symptoms:
There are two common types of atrioventricular canal defect — partial and complete. The partial form involves only the two upper chambers of the heart. The complete form allows blood to travel freely among all four chambers of the heart. In either type, extra blood circulates in the lungs.
Complete atrioventricular canal defect
Signs and symptoms of complete atrioventricular canal defect usually develop in the first several weeks of life. They include:
- Difficulty breathing (dyspnea)
- Lack of appetite
- Poor weight gain
- Bluish discoloration of the lips and skin (cyanosis)
- Fatigue
- Wheezing
- Swelling (edema) in the legs, ankles and feet
- Sudden weight gain from fluid retention
- Excessive sweating
- Decreased alertness
- Irregular or rapid heartbeat
Signs and symptoms of a partial atrioventricular canal defect may not appear until early adulthood. When they do become noticeable, signs and symptoms may be related to complications that develop as a result of the defect, and may include:
- Abnormal heartbeat (arrhythmia)
- Heart valve problems
- Heart failure
- High blood pressure in the lungs (pulmonary hypertension)
Contact your doctor if you or your child develops any of the following signs or symptoms. These could be indications of heart failure or another complication of atrioventricular canal defect:
- Poor appetite
- Failure to gain weight, in infants and children
- Shortness of breath
- Easily tiring
- Swelling of the legs, ankles or feet
- Bluish discoloration of the skin
Causes:
Atrioventricular canal defect occurs during fetal growth when your baby's heart is developing. While some factors, such as Down syndrome, may increase the risk of atrioventricular canal defect, in most cases the cause is unknown.
The normal-functioning heart
Your heart is divided into four chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout your body — your heart uses its left and right sides for different tasks. The right side moves blood into vessels that lead to your lungs. In your lungs, oxygen enriches your blood, which circulates to your heart's left side. The left side of your heart pumps blood into a large vessel called the aorta, which circulates blood to the rest of your body. Valves control the flow of blood into and out of the chambers of your heart. These valves open to allow blood to move to the next chamber or to one of the arteries, and they close to keep blood from flowing backward.
What happens in atrioventricular canal defect
In partial atrioventricular canal defect, a hole exists in the wall (septum) that separates the upper chambers (atria) of the heart. Also, the mitral valve between the upper and lower left chambers often has a defect called a cleft that causes it to leak (mitral valve regurgitation).
In complete atrioventricular canal defect, there's a large hole in the center of the heart where the walls between the upper chambers (atria) and lower chambers (ventricles) meet. Instead of two separate valves — one on the right (tricuspid) and one on the left (mitral) — one large common valve exists between the upper and lower chambers. And, this valve may not close tightly.
Oxygen-rich and oxygen-poor blood mix through the hole in the septum, and the abnormal valves leak blood into the heart's lower chambers (ventricles). These problems make the heart work harder, causing it to enlarge.
Complications:
Treating an atrioventricular canal defect helps your child avoid potential complications, such as:
- Enlargement of the heart. Increased blood flow through the heart forces it to work harder than normal, causing it to enlarge.
- Heart failure. Untreated, atrioventricular canal
defect will usually result in heart failure — a condition in which the
heart is unable to pump enough blood to meet the body's needs.
- High blood pressure in the lungs (pulmonary hypertension).
When the heart's left ventricle weakens and can't pump out enough
blood, the increase in pressure backs up through the pulmonary veins to
the arteries in the lungs, causing high blood pressure in the lungs.
- Pneumonia. If your baby has an untreated atrioventricular canal defect, he or she may have recurrent bouts of pneumonia — a serious lung infection.
Although treatment greatly improves the outlook for children with atrioventricular canal defect, some who have corrective surgery may still be at risk of the following associated conditions later in life:
- Leaky heart valves (regurgitation)
- Narrowing of the heart valves (stenosis)
- Heart rhythm abnormalities (arrhythmias)
- Breathing difficulties associated with damage to the lungs (pulmonary vascular disease)
Treatments and drugs:
Surgery is necessary to fix both complete and partial atrioventricular canal defects. During the procedure, the hole in the septum is closed using one or two patches. The patches remain in the heart permanently, becoming part of the septum as the heart's lining grows over it. The surgeon also
separates the one large heart valve between the upper and lower chambers into two separate valves.
For a partial atrioventricular canal defect, the surgery also involves repair of the mitral valve so it will close tightly. If repair isn't possible, the valve may need to be replaced instead.
If your baby has complete atrioventricular canal defect, the surgery also includes separation of the single valve into two valves, one on the left side and one on the right side of the repaired septum. If reconstruction of the single valve into two valves isn't possible, heart valve replacement may be necessary.
After surgery
If the atrioventricular canal defect has been successfully repaired with surgery, your child will likely lead a normal life, often with no activity restrictions.
However, your child will need lifelong follow-up care with a heart doctor (cardiologist) who specializes in congenital heart disease. The recommendation for follow-up is usually once a year, unless you have lingering problems, such as a leaky heart valve. In that case, follow-up will be more frequent.
Your child may also need to take preventive antibiotics before certain dental procedures and other surgical procedures if he or she is at risk of severe complications of endocarditis, a bacterial infection of the lining of the heart. Typically, this is when your child has some remaining defects after surgery, has received an artificial valve or has had repair with artificial (prosthetic) material.
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
When an atrioventricular canal defect has been surgically corrected before any permanent lung damage has occurred, women can generally expect to have normal pregnancies. Pregnancy is not recommended, however, if serious heart or lung damage occurred before surgery. An evaluation by a cardiologist is advised before women with repaired or unrepaired atrioventricular canal defect attempt pregnancy.
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