Ebstein’s Disease

What is Ebstein's Disease?

Ebstein’s disease is referred to as a defect of the heart that is rare and is present from birth. It is a kind of malformation in which some parts of the tricuspid valve are abnormal and do not work properly. Tricuspid valve is the valve that is situated between the two chambers of the heart, the right atrium and right ventricle. As a consequence of the damaged tricuspid valve, there may be some blood leakage from the valve, affecting the functioning of the heart. The size of the heart may further be enlarged, causing a heart failure. Quite frequently, this malformation may induce the tricuspid valve to forgo blood in a backward position into the right atrium, while the right ventricle squeezes. This backward flow increases the force in the right atrium which leaves the foramen ovale exposed. Such a condition causes deoxygenated blood to pass from the right atrium to the left atrium, moving directly to the parts of the body.

Ebstein’s disease is known as the deformity of the tricuspid valve and is marked by various factors such as the rearrangement of the functional annulus in a downward position, extension of the atrialised part of the right ventricle with some degree of thinning of the wall, dismissal of the frontal leaflet, and expansion of atrioventricular junction.

Symptoms

The moderate types of Ebstein’s disorder might show symptoms at a later stage in life. The symptoms that manifest in a person may include:

  • Breathlessness because of great exertion.
  • Excessive exhaustion.
  • Abnormal rhythms of the heart and heart palpitations.
  • Discolouration of the skin and lips because of cyanosis
  • Chronic cough.
  • Inability to grow.
  • Breathing rapidly.

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Causes

A child has this defect from birth, and why it occurs is unknown. The main reason is the deformity in the tricuspid valve which is made of three leaflets.

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Risks

As the disease develops at a very early stage of the baby’s heart development, the factors that affect the disease are unknown. However, certain factors can be considered as risks for development of such defects:

  • Environmental factors.
  • Genetic factors.
  • Exposure to lithium during pregnancy.
  • Mother suffering viral infection during pregnancy.

 

 

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Prevention

There is no known prevention of the defect, but one can take proper precautions during pregnancy to avoid any such probability. Ask your provider before taking medicines, if they are considered to be related to the development of the disease.

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How is it diagnosed?

In order to diagnose this disease, our doctor at Medanta might recommend the following tests: 

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How is it treated?

The treatment of this disease depends on the extent of the intensity. The purpose of the treatment is to lessen the symptoms, not giving chance to any future complications to develop. At Medanta, our specialists may employ various treatment method..

  • Regular monitoring

    If no symptoms of the disease is seen, then the doctor might recommend cautious monitoring of the heart’s condition followed by regular checkups.

  • Tests and physical examination

    Tests like echocardiogram, electrocardiogram, and stress test could be really helpful.

  • Medications

    If disturbances with the heart’s rhythm are detected, medications might help control the heart rate, and improve rhythm of the heart. Drugs like diuretics will help prevent the retention of water in the body. If there is trouble with rhythm of the heart, medicines might be recommended to prevent blood clots between the heart chambers.

  • Surgery

    Surgery for this disorder is recommended if you have moderate symptoms, but there is enlargement of the heart and decrease in its functioning. This condition is of rare nature, therefore an expert surgeon should be consulted.

When do I contact the doctor?

You need to consult a doctor in case of breathlessness during exertion, abnormal rhythm of the heart, or palpitations. If your child suffers discolouration of the skin or there is a delayed growth, you need to consult a doctor and know exact reasons.

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