Ebstein's anomaly
Definition:
Ebstein's anomaly is a rare heart defect that's present at birth (congenital). In Ebstein's anomaly, your tricuspid valve — the valve between the chambers on the right side of your heart — doesn't work properly. Blood leaks back through the valve, making your heart work less efficiently. Ebstein's anomaly may also lead to enlargement of the heart or heart failure.
If you have no signs or symptoms associated with Ebstein's anomaly, careful monitoring of your heart may be all that's necessary. If signs and symptoms bother you, or if the heart is enlarging or becoming weaker, treatment for Ebstein's anomaly may be necessary. Treatment options include medications and surgery.
Symptoms:
Mild forms of Ebstein's anomaly may not cause symptoms until later in adulthood. If signs and symptoms are present, they may include:
If you or your child has signs or symptoms of heart failure — such as feeling easily fatigued or short of breath, even with normal activity — or is showing blue skin coloration around the lips and nails (cyanosis), talk to your doctor. He or she may refer you to a doctor who specializes in heart disease (cardiologist).
Causes:
Ebstein's anomaly is a heart defect that you have at birth (congenital). Why it occurs is still unknown. To understand how Ebstein's anomaly affects your heart, it helps to know a little about how the heart works to supply your body with blood.
How your heart works
Your heart is made up of four chambers. The top two chambers are called the right and left atria (plural for atrium). The two lower chambers, the ventricles, are larger, thicker walled chambers that do the work of pumping blood. Separating the atria from the ventricles are valves, which keep the blood flowing in the right direction through the heart. Each valve consists of two or three strong, thin flaps (leaflets) of tissue. When closed, a valve prevents blood from flowing to the next chamber or from returning to the previous chamber.
Blood returning from your body, which lacks oxygen, flows into the right atrium, through the tricuspid valve and then into the right ventricle, which pumps the blood to your lungs to receive oxygen. On the other side of your heart, oxygen-rich blood from your lungs flows into the left atrium, through the mitral valve and then into the left ventricle, which then pumps the blood to the rest of your body.
What happens in Ebstein's anomaly
In Ebstein's anomaly, the tricuspid valve is farther down into the right ventricle than it would be in a normal heart. This makes it so that a portion of the right ventricle becomes part of the right atrium (becomes atrialized), causing the right atrium to be larger than usual. Because of this, the right ventricle can't work properly.
In addition to the problems with the placement of the tricuspid valve, the valve's leaflets are abnormally formed. This can lead to blood leaking backward (regurgitating) into the right atrium. In severe cases, the leaflets may be tethered to the wall of the heart, leading to severe leakage of blood into the atrium.
Where the valve is placed and how poorly it's formed varies from person to person. In some people, the valve is only mildly abnormal. In others, the valve may be extremely displaced, and it may leak severely. The more the valve leaks, the more the right atrium enlarges as it receives more blood. At the same time, the right ventricle enlarges (dilates) as it tries to cope with the leaky valve and still deliver blood to the lungs. Thus, the right-sided chambers of the heart enlarge, and as they do, they weaken, leading to heart failure.
Other heart conditions associated with Ebstein's anomaly
Other heart conditions may be associated with Ebstein's anomaly. Three common conditions are:
Complications:
Many people with mild Ebstein's anomaly have few complications. However, you may need to take some precautions in certain situations:
Treatments and drugs:
Treatment of Ebstein's anomaly depends on the severity of the defect and your signs and symptoms. The goal of treatment is to reduce your symptoms and avoid future complications, such as heart failure and arrhythmias. Treatments may include:
Regular monitoring
If you have no signs or symptoms or abnormal heart rhythms, your doctor may recommend only careful monitoring of your heart condition with regular checkups. Checkups typically include a physical exam, electrocardiogram, chest X-ray, echocardiogram and, if necessary, a Holter monitor test. Your doctor may also ask you to undergo an exercise test, such as walking on a treadmill. This test checks your heart's response to physical activity.
Medications
If you have heart rhythm disturbances, medications may help control heart rate and maintain normal heart rhythm. Doctors commonly use medications, including calcium channel blockers, beta blockers, digitalis and anti-arrhythmic drugs, such as amiodarone.
Your doctor may also prescribe medications for signs and symptoms of heart failure, if you need them. These may include diuretics and other medications.
Surgical heart repair
Your doctor may recommend surgical repair when your signs and symptoms are affecting your quality of life or when your heart begins to enlarge and overall heart function begins to decrease. Because Ebstein's anomaly is rare, choose a surgeon who's familiar with the defect and who has experience performing procedures to correct it. Different types of procedures can be used to surgically treat Ebstein's anomaly and associated defects, including:
Radiofrequency catheter ablation is another procedure that can help treat a fast heartbeat or WPW syndrome. In this procedure, one or more catheters are threaded through your blood vessels to your inner heart, where the electrical impulses that cause your heart to beat are generated. Electrodes at the catheter tips are heated with radiofrequency energy. This destroys (ablates) a small spot of heart tissue and creates an electrical block along the pathway that's causing your arrhythmia. Usually, this stops your arrhythmia. However, this procedure may be less effective for treating people with Ebstein's anomaly than for those with other heart problems. Repeat procedures may be necessary.
Heart transplantation
If you have severe Ebstein's anomaly and poor heart function, a heart transplant may be necessary.
Definition:
Ebstein's anomaly is a rare heart defect that's present at birth (congenital). In Ebstein's anomaly, your tricuspid valve — the valve between the chambers on the right side of your heart — doesn't work properly. Blood leaks back through the valve, making your heart work less efficiently. Ebstein's anomaly may also lead to enlargement of the heart or heart failure.
If you have no signs or symptoms associated with Ebstein's anomaly, careful monitoring of your heart may be all that's necessary. If signs and symptoms bother you, or if the heart is enlarging or becoming weaker, treatment for Ebstein's anomaly may be necessary. Treatment options include medications and surgery.
Symptoms:
Mild forms of Ebstein's anomaly may not cause symptoms until later in adulthood. If signs and symptoms are present, they may include:
- Shortness of breath
- Fatigue, especially with exertion
- Leg swelling
- Heart palpitations or abnormal heart rhythms (arrhythmias)
- A bluish discoloration of the lips and skin caused by low oxygen (cyanosis)
If you or your child has signs or symptoms of heart failure — such as feeling easily fatigued or short of breath, even with normal activity — or is showing blue skin coloration around the lips and nails (cyanosis), talk to your doctor. He or she may refer you to a doctor who specializes in heart disease (cardiologist).
Causes:
Ebstein's anomaly is a heart defect that you have at birth (congenital). Why it occurs is still unknown. To understand how Ebstein's anomaly affects your heart, it helps to know a little about how the heart works to supply your body with blood.
How your heart works
Your heart is made up of four chambers. The top two chambers are called the right and left atria (plural for atrium). The two lower chambers, the ventricles, are larger, thicker walled chambers that do the work of pumping blood. Separating the atria from the ventricles are valves, which keep the blood flowing in the right direction through the heart. Each valve consists of two or three strong, thin flaps (leaflets) of tissue. When closed, a valve prevents blood from flowing to the next chamber or from returning to the previous chamber.
Blood returning from your body, which lacks oxygen, flows into the right atrium, through the tricuspid valve and then into the right ventricle, which pumps the blood to your lungs to receive oxygen. On the other side of your heart, oxygen-rich blood from your lungs flows into the left atrium, through the mitral valve and then into the left ventricle, which then pumps the blood to the rest of your body.
What happens in Ebstein's anomaly
In Ebstein's anomaly, the tricuspid valve is farther down into the right ventricle than it would be in a normal heart. This makes it so that a portion of the right ventricle becomes part of the right atrium (becomes atrialized), causing the right atrium to be larger than usual. Because of this, the right ventricle can't work properly.
In addition to the problems with the placement of the tricuspid valve, the valve's leaflets are abnormally formed. This can lead to blood leaking backward (regurgitating) into the right atrium. In severe cases, the leaflets may be tethered to the wall of the heart, leading to severe leakage of blood into the atrium.
Where the valve is placed and how poorly it's formed varies from person to person. In some people, the valve is only mildly abnormal. In others, the valve may be extremely displaced, and it may leak severely. The more the valve leaks, the more the right atrium enlarges as it receives more blood. At the same time, the right ventricle enlarges (dilates) as it tries to cope with the leaky valve and still deliver blood to the lungs. Thus, the right-sided chambers of the heart enlarge, and as they do, they weaken, leading to heart failure.
Other heart conditions associated with Ebstein's anomaly
Other heart conditions may be associated with Ebstein's anomaly. Three common conditions are:
- Atrial septal defect. About half the people with
Ebstein's anomaly have a hole between the two upper chambers of the
heart called an atrial septal defect (ASD). This hole may allow
deoxygenated blood in the right atrium to mix with oxygenated blood in
the left atrium, decreasing the amount of oxygen available in your
blood. This causes a bluish discoloration of the lips and skin
(cyanosis). The ASD associated with Ebstein's anomaly can increase your
risk of a blood clot passing from the veins in your heart into the blood
vessels leading to your brain and causing a stroke. If you have surgery
to repair your tricuspid valve, your surgeon will also close the ASD at
the same time.
- Abnormal heartbeats (arrhythmias). Some people with
Ebstein's anomaly have an abnormal heart rhythm (arrhythmia)
characterized by rapid heartbeats (tachycardia). These types of
arrhythmias (tachyarrhythmias) can make your heart work less
effectively, especially when the tricuspid valve is leaking severely. In
some cases, a very fast heart rhythm may cause fainting spells
(syncope).
- Wolff-Parkinson-White (WPW) syndrome. Some people with Ebstein's anomaly may also have a condition known as Wolff-Parkinson-White syndrome — an abnormal electrical pathway in the heart. The presence of WPW syndrome can lead to very fast heart rates and fainting spells.
Complications:
Many people with mild Ebstein's anomaly have few complications. However, you may need to take some precautions in certain situations:
- Being active. If you have mild Ebstein's anomaly
with a nearly normal heart size and no heart rhythm disturbances, you
can probably participate in most physical activities. Depending on your
signs and symptoms, your doctor may recommend that you avoid competitive
sports, such as football or basketball. Your doctor can help you decide
which activities are right for you.
-
During pregnancy. In many cases, women with mild
Ebstein's anomaly can safely have children. But pregnancy does have
risks. If you plan on becoming pregnant, be sure to talk to your doctor
ahead of time. He or she can tell you if it's safe for you to become
pregnant and help decide how much extra monitoring you may need
throughout pregnancy and childbirth.
Being pregnant puts additional strain on your heart and circulatory system not only during pregnancy, but also during labor and delivery and for a period of time after your baby's birth. There is an increased risk of stroke if you have a hole between the upper chambers of the heart (atrial septal defect). Rarely, women with Ebstein's anomaly develop severe complications that can cause death during pregnancy.
Treatments and drugs:
Treatment of Ebstein's anomaly depends on the severity of the defect and your signs and symptoms. The goal of treatment is to reduce your symptoms and avoid future complications, such as heart failure and arrhythmias. Treatments may include:
Regular monitoring
If you have no signs or symptoms or abnormal heart rhythms, your doctor may recommend only careful monitoring of your heart condition with regular checkups. Checkups typically include a physical exam, electrocardiogram, chest X-ray, echocardiogram and, if necessary, a Holter monitor test. Your doctor may also ask you to undergo an exercise test, such as walking on a treadmill. This test checks your heart's response to physical activity.
Medications
If you have heart rhythm disturbances, medications may help control heart rate and maintain normal heart rhythm. Doctors commonly use medications, including calcium channel blockers, beta blockers, digitalis and anti-arrhythmic drugs, such as amiodarone.
Your doctor may also prescribe medications for signs and symptoms of heart failure, if you need them. These may include diuretics and other medications.
Surgical heart repair
Your doctor may recommend surgical repair when your signs and symptoms are affecting your quality of life or when your heart begins to enlarge and overall heart function begins to decrease. Because Ebstein's anomaly is rare, choose a surgeon who's familiar with the defect and who has experience performing procedures to correct it. Different types of procedures can be used to surgically treat Ebstein's anomaly and associated defects, including:
- Tricuspid valve repair. This involves reducing the
size of the valve opening and allowing the existing valve leaflets to
come together to work properly. This procedure is usually done when
there's enough valve tissue to allow for repair.
- Tricuspid valve replacement. If the existing valve
can't be repaired, it may be replaced by removing the deformed valve and
inserting either a mechanical valve or a specially treated biological
tissue (bioprosthetic) valve. If a mechanical valve is used, you'll need
to take a blood-thinning medication for the rest of your life.
- Closure of the atrial septal defect (ASD). If an ASD is present, your surgeon can close the hole during surgery to repair or replace the defective valve.
- Maze procedure. Another procedure that doctors may perform during valve repair or replacement surgery is called a maze procedure. It's used to help correct fast heart rhythms in the atrium (atrial tachyarrhythmias). To do the procedure, your surgeon makes a series of incisions in the right atrium with a scalpel or a device that destroys tissue by freezing it. When these incisions heal, the scars create a barrier of tissue in your heart that causes it to beat at a normal rate. The Maze procedure is an open-heart surgery.
Radiofrequency catheter ablation is another procedure that can help treat a fast heartbeat or WPW syndrome. In this procedure, one or more catheters are threaded through your blood vessels to your inner heart, where the electrical impulses that cause your heart to beat are generated. Electrodes at the catheter tips are heated with radiofrequency energy. This destroys (ablates) a small spot of heart tissue and creates an electrical block along the pathway that's causing your arrhythmia. Usually, this stops your arrhythmia. However, this procedure may be less effective for treating people with Ebstein's anomaly than for those with other heart problems. Repeat procedures may be necessary.
Heart transplantation
If you have severe Ebstein's anomaly and poor heart function, a heart transplant may be necessary.
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2 komentar:
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