Ebstein's aberration or anomaly is one of the rare heart disorders, in which the tricuspid valve present between the atrium and ventricle of the right heart does not work properly. It cannot prevent blood from leaking back through the valve and thus, the right side of the heart is not able to work efficiently. It results in unusual enlargement of right heart and may also lead to failure of heart. Ebstein's aberration or anomaly defect is present in an individual from the birth.

Causes of Ebstein's Aberration or Anomaly

Causes of Ebstein's Aberration or Anomaly

The exact causes of Ebstein's aberration or anomaly are still not known. The only thing that is seen is that it is a congenital condition. Researchers are still trying to find more about this medical condition and what causes this condition.

Can Intake of Lithium During Pregnancy Cause Ebstein's Aberration or Anomaly?

Lithium may not cause Ebstein's aberration or anomaly, but lithium intake during pregnancy is a factor of risk for Ebstein's aberration or anomaly.

Symptoms of Ebstein's Aberration or Anomaly

An individual suffering from Ebstein's aberration or anomaly does not necessarily experience the symptoms unless it is a severe case of it. The common signs and symptoms of Ebstein's aberration or anomaly are as follows:

  • Lack of stamina.
  • Frequent exertion with shortness of breath is a symptom of Ebstein's aberration or anomaly.
  • Reduced level of oxygen in the body leading to skin and lips turning bluish.
  • Unusual Rhythms of Heart also called as Heart palpitations.

Tests to Diagnose Ebstein's Aberration or Anomaly

The diagnosis of Ebstein's aberration or anomaly starts with a routine physical test by a medical expert. If the signs of symptoms associated with the medical condition suggests a possibility of Ebstein's aberration or anomaly, the doctor suggests one or more of the following tests to confirm the condition.

  • Echocardiogram is used to detect Ebstein's aberration or anomaly. It is a common test that is used to detect congenital heart problems. In this test, sound waves are used to create an image of the heart and look for any signs of abnormalities in the structure or functioning of the heart. Transesophageal echocardiogram is also used in some cases to get a better view of the heart. It is a special type of echocardiogram, in which the sound producing transducer attached to a narrow tube is inserted into your food pipe through your throat. As the food pipe is located close to the heart, it gives a better image of the heart.
  • Another test for Ebstein's aberration or anomaly is electrocardiogram (ECG). It is a special test which makes use of electrical signals to detect any problems with the structure or working of your heart. This test is conducted by attaching electrodes to multiple points over your heart to monitor for the heart beat and its characteristics.
  • Chest X-ray are also helpful in diagnosing the condition of Ebstein's aberration or anomaly indicated by enlarged size of the right heart in the X-ray.
  • A cardiac MRI is an advanced method of analyzing the structure of heart. In this test, electromagnetic waves are used to create an image of the heart. The image of the heart created by this method helps in identifying any abnormality in the structure or functioning of heart.
  • Holter monitor is a special type of ECG, which looks for any disturbances in the heart beats. The device fitted into your body and it observes the electrical signals that are used for analysis of heart condition.
  • Pulse oximetry tests for diagnosis of Ebstein's aberration or anomaly are used to analyze the amount of oxygen present in your blood as lack of oxygen is also an indication of the possible heart problems. The analysis is done with the help of a sensor that is attached to the tip of the fingers of your legs.
  • Exercise stress test for Ebstein's aberration or anomaly are used to analyze the behavior of your heart when your body is undergoing heavy physical exercises that causes exertion. You are asked to ride a stationary bicycle or walk on a treadmill while different parameters of the heart are monitored closely. This test is also used to identify the amount of exertion that your body can bear without any problem.

Treatment for Ebstein's Aberration or Anomaly

There are various types of treatment options available for Ebstein's aberration or anomaly. The choice of a treatment option is mainly based of the severity of the symptoms and underlying heart problems. The treatment options cannot eliminate the problem completely, but can reduce the chances of complications such as cardiac arrest or heart failure. Various treatment options for Ebstein's aberration or anomaly are as follows:

  1. Regular Monitoring of Ebstein's Aberration or Anomaly

    A close and regular monitoring of the condition of the heart is sufficient if the patient does not show any signs or symptoms of abnormal heart condition. The doctor would examine your condition at a regular interval with the help of certain tests to make sure that there are no serious problems. The tests conducted during the routine visits include a Holter monitor test, echocardiogram, electrocardiogram, and an exercise stress test.

  2. Treating Ebstein's Aberration or Anomaly with Medication

    Some of the heart problems such as abnormal heart rate, heartbeat in the case of Ebstein's aberration or anomaly can be controlled by certain medications. Medications are helpful to prevent water retention (diuretics) during heart failure or cardiac arrest. Medications are also helpful with heart problems such as unusual heart beat.

    In case of Ebstein's aberration or anomaly, there is also a risk of blocking of pathway that connects heart with pulmonary artery and aorta in cases of infants. This situation can easily cause reduced flow of blood in the body. Medications like nitric oxide is used to avoid any complications in such cases.

  3. Surgery for Ebstein's Aberration or Anomaly

    Surgery for treating Ebstein's aberration or anomaly is always kept as the last treatment option just like in most other medical complications as surgery itself has its complications. Surgery is usually preferred when other treatment options do not show any signs of improvements and your condition continues to get poor. If you are looking for surgery, it is important to choose a specialist surgeon as it is a rare condition and any imperfection can be fatal.

Life Expectancy for Ebstein's Aberration or Anomaly

The life expectancy for women with Ebstein's aberration or anomaly is better than men. The life expectancy of women with Ebstein's aberration or anomaly is 39 years and for men is 37 years.

Prognosis/ Outlook of Ebstein's Aberration or Anomaly

The prognosis or outlook for Ebstein's aberration or anomaly is not good. Very few patients with Ebstein's aberration or anomaly survive more than 50 years. The prognosis for Ebstein's aberration or anomaly depends on many factors, which includes the severity of tricuspid reflux, related arrhythmias and the related anatomical defects.

Risk Factors for Ebstein's Aberration or Anomaly

Ebstein's aberration or anomaly is a congenital heart defect and thus, it can be caused due to genetic defect as well as environmental factors. The risk increases in case of a medical history of heart disorders in the family. Intake of certain medications like lithium by a woman during pregnancy can also increase the risk of developing Ebstein's aberration or anomaly in the child.

Complications of Ebstein's Aberration or Anomaly

Complications are not usually observed in mild cases of Ebstein's aberration or anomaly. However, an individual needs to be careful in severe cases this illness:

  • Exertion due to sports activities can complicate Ebstein's aberration or anomaly. A person suffering from severe form of Ebstein's aberration or anomaly need to watch out for physical activities that may cause exertion and related problems. Sports activities basket, football, etc., may need to be avoided as per doctor’s instructions.
  • Pregnancy related complications are seen in this condition. A women suffering from Ebstein's aberration or anomaly can become pregnant and give birth to a child, but there are various forms of risk associated with pregnancy. A women suffering from Ebstein's aberration or anomaly needs special care and monitoring during pregnancy. Also, there are chances of complications during labor as it requires lots of stamina and increased circulation of blood.
  • In rare cases, Ebstein's aberration or anomaly may also result in problems related to heart rhythm and even cause heart failure or cardiac arrest.

Coping with Ebstein's Aberration or Anomaly

Mild cases of Ebstein's aberration or anomaly does not have a significant impact on the life of an individual. However, serious cases of Ebstein's aberration or anomaly needs proper care and attention, especially in children. Following methods can help an individual in coping up from the problem.

  • Follow the Instructions of the doctor carefully for proper coping with Ebstein's aberration or anomaly. If you have a condition of Ebstein's aberration or anomaly and is interrupting with your routine activities, you need to see your doctor as soon as possible. Most of the problems related to the heart can kept under control only if you follow the instructions of the doctor and take all the medications in time. Also, you must report any issues to the doctor immediately.
  • For proper coping with Ebstein's aberration or anomaly, take medications in time. It is very important for someone suffering from Ebstein's anomaly to take all the medication in time without any change in dosage of frequency. Medications are helpful in easing out the symptoms that may interfere your normal routine activities.
  • Staying active is also an excellent coping strategy. With one medical condition, the chances of other problems also increases. Thus, it is necessary for a person suffering from the medical condition of Ebstein's aberration or anomaly to include exercise in the daily routine. It helps in keeping your body healthy with proper blood circulation and better stamina. In case of children, he or she must be encouraged to be active and take part in physical activities with some precautions.
  • Support groups are also a way to cope. A person suffering from Ebstein's aberration or anomaly can also join a support groups where there are experts to offer good counseling. This makes you more aware of the disease and you also learn how to cope up with the symptoms of Ebstein's aberration or anomaly.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: April 14, 2017

Pain Assist Inc.

Pramod Kerkar

Views: 231

  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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