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Ebstein's anomaly

Definition


Disease: Ebstein's anomaly Ebstein's anomaly
Category: Cardiovascular diseases

Disease Definition:

Ebstein’s anomaly is a congenital and rare heart defect, in which the tricuspid valve, which is the valve between the chambers on the right side of the heart, doesn’t work properly. In this case, blood leaks back through the valve and makes the heart work less efficiently. This disease could also cause heart failure or enlargement of the heart.

 

Careful monitoring of the heart could be the only necessary thing in case a person doesn’t have any signs or symptoms of Ebstein’s anomaly. However, treatment for Ebstein’s anomaly may be necessary in case the signs and symptoms become troublesome or if the heart is enlarging or becoming weaker. Medications and surgery are some of the available treatment options.
 

Work Group:


Symptoms, Causes

Symptoms:

In case someone has a mild form of Ebstein’s anomaly, they may not experience any signs or symptoms until later in adulthood. Some people with very abnormal valves might have minimal problems, if any.

 

The signs and symptoms of Ebsteins’ anomaly develop slowly over many years and may include:

 

  • Leg swelling
  • Shortness of breath
  • Fatigue, particularly with exertion
  • Arrhythmia or heart palpitations
  • Cyanosis, which is the bluish discoloration of the lips and skin caused by low oxygen.

 

Early signs of cyanosis and heart failure could be present in newborns and infants who are diagnosed with Ebstein’s anomaly. Usually, a severe defect that requires treatment makes signs and symptoms appear at such a young age.

 

Signs and symptoms of heart failure, such as tiring easily or becoming short of breath during play occur in older children. Heart pounding or racing or cyanosis, particularly around the lips and fingertips could be some of the other signs and symptoms of older children.

 

A person should talk to a doctor who will probably refer them to a cardiologist in case they or their child:
Shows signs and symptoms of heart failure, including feeling easily fatigued or short of breath, even with normal activity.
Has cyanosis, which is the blue skin coloration around the lips and nails.
 

Causes:

This disease is a congenital heart defect.
Here is some information on how the heart works to supply the body with blood:

 

The heart has four chambers. The top two chambers are called atria, and the two lower chambers are called ventricles, which are larger and thicker walled chambers that do the work of pumping blood. Valves separate the atria from the ventricles and keep the blood flowing in the right direction through the heart. Each valve consists of two or three strong and thin leaflets of tissue. When a valve is closed, blood is prevented from flowing to the next chamber or from returning to the previous chamber.

 

Blood poor with oxygen returns from the body and flows into the right atrium through the tricuspid valve and then into the right ventricle that pumps the blood to the lungs and receives oxygen. On the other side of the heart, blood rich with oxygen flows from the lungs into the left atrium through the mitral valve and then into the left ventricle that pumps the blood to the rest of the body.

 

In Ebstein’s anomaly, the tricuspid is placed farther down into the right ventricle than it would normally be, making it so that a portion of the right ventricle becomes part of the right atrium or becomes atrialized and causes the right atrium to be larger than usual. The right ventricle can’t work properly because of all this.

 

Also, the valve’s leaflets are abnormally formed, which can lead to blood leaking backward into the right atrium. The leaflets could be tethered to the wall of the right heart in some severe cases, which inhibits their movement and leads to severe leakage of blood into the atrium. Usually, one leaflet is enlarged.

 

The abnormal place of the valve and its poor form differs from one person to another. In some cases, the valve could be only mildly abnormal. However, in other cases, the valve could be extremely displaced and malformed, and may leak severely. The more the valve leaks, the more the right atrium enlarges as it receives more blood. During this time, the right ventricle enlarges as it tries to cope with the leaky valve and still deliver blood to the lungs. Because of this, the right-sided chambers of the heart enlarge and weaken, causing heart failure.

 

There are three common conditions that are associated with Ebstein’s anomaly:

Arrhythmias:

Some people suffering from this disease could have an abnormal heart rhythm that is characterized by rapid heartbeats, a condition called tachycardia. These arrhythmias could cause the heart to work less effectively, particularly when there’s severe leakage in the tricuspid valve. Sometimes, syncope (fainting spells) could be caused due to a very fast heart rhythm.

Atrial septal defect:

Almost half the people with this disease have ASD (atrial septal defect), a hole between the two upper chambers of the heart, which could allow deoxygenated blood in the right atrium to mix with oxygenated blood in the left atrium and thus, decrease the amount of oxygen available in the blood. The result of this is cyanosis, which is the bluish discoloration of the lips and skin. When ASD is associated with Ebstein’s anomaly, it could increase a person’s risk of a blood clot passing from the veins in the heart into the blood vessels that lead to the brain and cause a stroke. In case a person’s having surgery in order to repair the tricuspid valve, their ASD will also be closed at the same time.

Wolff-Parkinson-White (WPW) syndrome:

In some cases, people with Ebstein’s anomaly might have an abnormal electrical pathway in their heart known as Wolff-Parkinson-White syndrome, which could lead to very fast heart rates and fainting spells.
 

Complications

Complications:

Usually, people with this disease have only a few complications, but in certain situations they need to take some precautions. These situations include:

Pregnancy:

Women can safely have children in most cases of mild Ebstein’s anomaly. However, pregnancy has its risks. During pregnancy, labor, delivery and for a period of time after the baby’s birth additional strain is put on the heart and circulatory system. In case a woman has a hole between the upper chambers of her heart (ASD), it means that she has an increased risk of a stroke. In some rare cases, Ebstein’s anomaly could cause death due to severe complications. A woman should talk to her cardiologist before becoming pregnant, because the doctor can tell her whether it’s safe to become pregnant and help decide how much extra monitoring she may need throughout pregnancy and childbirth.

Being active:

A person can participate in most physical activities in case their Ebstein’s anomaly is mild, the size of their heart is nearly normal and they don’t have any heart rhythm disturbances. They may be recommended avoiding competitive sports, including football and basketball, depending on their signs and symptoms. In order to decide which activities are right for them, the patient should talk to a cardiologist.

 

Heart failure, rhythm problems and in some rare cases, sudden cardiac arrest or stroke are some of the other complications that could result from Ebstein’s anomaly.
 

Treatments:

Treatment for Ebstein’s anomaly will depend on the severity of the defect as well as its signs and symptoms. Reducing symptoms and avoiding future complications, such as heart failure and arrhythmias are some of the goals of treatment. They may include:

 

REGULAR MONITORING:

A person may be recommended only careful monitoring of their heart condition along with regular checkups in case they have no signs and symptoms or abnormal heart rhythms. Checkups include a physical exam, chest X-ray, electrocardiogram, echocardiogram and in some cases, a Holter monitor test. The patient may also be asked to undergo an exercise test, such as walking on a treadmill, which will check their heart’s responses to physical activity.

 

MEDICATIONS:

Some medications could help control heart rate and maintain normal heart rhythm in case a person has heart rhythm disturbances. Beta blockers, calcium channel blockers, digitalis and anti-arrhythmic drugs, such as amiodarone are some of the commonly used medications.
The patient may also be prescribed diuretics and other medications for signs and symptoms of heart failure.

 

SURGICAL HEART REPAIR:

If someone’s signs and symptoms are troublesome, or if their heart begins to enlarge and overall heart functions begins to decrease, they may be recommended surgical repair. The patient should choose a doctor who’s familiar with the defect and has experience performing procedures to correct it, because Ebstein’s anomaly is a rare disease. In order to surgically repair Ebstein’s anomaly and other associated defects different types of procedures could be used, such as:

Tricuspid valve repair:

Usually, this procedure is done when there’s enough valve tissue to allow for repair. This procedure involves reducing the size of the opening of the valve and allowing the existing valve leaflets to come together to work properly.

 

Tricuspid valve replacement: This procedure is done when the existing valve cannot be repaired. During this procedure the deformed valve will be removed, and in its place either a mechanical valve or a specially treated biological tissue (bioprosthetic) valve will be inserted. However, the patient will need to take a blood-thinning medication for the rest of their life in case a mechanical valve is inserted.

Radiofrequency catheter ablation:

This procedure can help treat a fast heartbeat or WPW syndrome. During this procedure, one or more catheters are threaded through the blood vessels to the inner heart where the electrical impulses that cause the heart to beat are generated. The electrodes that are at the tips of the catheters are heated with radiofrequency energy. A small spot of heart tissue is destroyed and an electrical block is created along the pathway that is causing the arrhythmia. This procedure usually stops the arrhythmia.

Maze procedure:

This procedure is an open heart surgery. A maze procedure, which is used to help correct fast heart rhythms in the atrium (atrial tachyarrhythmias), could also be performed during valve repair or replacement surgery. During this procedure, a series of incisions are made in the right atrium with a scalpel or a device that destroys tissue by freezing it. After those incisions heal, the scars will create a barrier of tissue in the heart, causing it to beat at a normal rate.

 

Closure of the ASD (atrial septal defect): During surgery to repair or replace the defective valve, the ASD could also be closed if the patient has one.

 

HEART TRANSPLANTATION:

In some cases, heart transplantation could be necessary in case the patient Ebstein’s anomaly with a severely malformed valve and poor heart function.
 

Prognosis:

Not available

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