Ebstein’s anomaly

Ebstein’s anomaly. Ebstein’s anomaly (also called “Ebstein’s malformation”) is a defect that mainly affects the tricuspid valve. This valve separates the lower right chamber of the heart (right ventricle) from the upper right chamber (right atrium). It is a congenital condition, which means it is present from birth.


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  • 1 Causes, incidence and risk factors
  • 2 Symptoms
  • 3 Signs and tests
  • 4 Treatment
  • 5 Forecast
  • 6 Complications
  • 7 Prevention
  • 8 Source

Causes, incidence and risk factors

The tricuspid valve is normally made up of three parts, called leaflets or flaps. The leaflets open to allow blood to move from the right atrium (upper chamber) to the right ventricle (lower chamber) while the heart relaxes. They close to prevent blood from moving from the right ventricle to the right atrium while the heart is pumping.

In people with Ebstein’s abnormality, the leaflets are unusually deep in the right ventricle and are often larger than normal. The defect usually causes the valve to malfunction and blood can go the wrong way back to the right atrium. Holding back the blood flow can lead to swelling of the heart and fluid buildup in the lungs or liver . Sometimes blood cannot flow from the heart into the lungs, and the person may take on a blue appearance.. In most cases, patients also have a hole in the wall that separates the two upper chambers of the heart, and circulation through this hole can cause deoxygenated blood to flow into the body.

Also, there may be narrowing of the valve that leads to the lungs (pulmonary valve). Ebstein’s abnormality occurs as a baby develops in the womb . The exact cause is unknown, although the use of certain drugs (such as lithium or benzodiazepines) during pregnancy may play a role. The condition is rare and more common in white people.


Symptoms range from mild to very severe. Symptoms often develop immediately after birth and include bluish-colored lips and nails due to low levels of oxygen in the blood . In severe cases, the baby seems very sick and has trouble breathing.

Symptoms in older children may include:

  • Insufficiency to grow.
  • Fast breathing.
  • Difficulty breathing.
  • Very fast heartbeat.

Signs and tests

Newborns who have a severe leak through the tricuspid valve will have very low levels of oxygen in their blood and considerable swelling of the heart . The doctor may hear abnormal heart sounds, such as a murmur, when auscultating the chest with a [stethoscope]]. Tests that can help diagnose this condition include:

  • Chest x -ray.
  • Magnetic resonance imaging (MRI) of the heart.
  • Measurement of the electrical activity of the heart (ECG).
  • Ultrasound of the heart (echocardiography).


Treatment depends on the severity of the defect and the specific symptoms. Medical care may include:

  • Medicines to help with heart failure.
  • Oxygen and other respiratory support.
  • Surgery may be needed to correct the valve for children who continue to get worse or who have more serious complications.


In general, the earlier symptoms appear, the more severe the disease . Some patients may not have any symptoms or have very mild symptoms. Others may worsen over time: they have a bluish discoloration ( cyanosis ), heart failure, atrioventricular block, or dangerous heart rhythms.


A severe leak can lead to swelling of the heart and liver , as well as congestive heart failure.

Other complications may include:

  • Abnormal heart rhythms (arrhythmias), including abnormally fast rhythms (tachyarrhythmias) and abnormally slow rhythms (bradyarrhythmias and atrioventricular block).
  • Clots of blood from the heart to other parts of the body.
  • Brain abscess


There is no known form of prevention other than talking to your doctor before pregnancy if you are taking medications that are believed to be related to the development of this disease. You can prevent some of the complications of the disease. For example, taking antibiotics before dental surgery can help prevent endocarditis.

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