Ebstein Anomaly

An uncommon birth cardiac abnormality is called the Ebstein anomaly (congenital). Your tricuspid valve is misaligned in this situation, and the valve's leaflets are deformed. The valve does not function correctly as a result.

Your heart may perform less effectively as a result of blood leaking back through the valve. Heart failure and heart enlargement are other complications of the Ebstein anomaly.

Regular cardiac monitoring may be sufficient if you don't exhibit any Ebstein abnormality symptoms or indications. If the signs and symptoms irritate you or if your heart is expanding or weakening, you may require treatment. Surgery and medication are available as treatment options.

Ebstein Anomaly: Causes

You are born with a heart defect called an Ebstein anomaly (congenital). There is no known cause. Understanding how the heart pumps blood throughout your body can help you comprehend how the Ebstein anomaly affects your heart.

Normal placement of the tricuspid valve is between the two right heart chambers (right atrium and right ventricle).

Tricuspid valve positioning in the right ventricle is abnormal in Ebstein abnormality. As a result, a section of the right ventricle merges with the right atrium, expanding it and impairing its functionality.

The leaflets of the tricuspid valve are also malformed. Blood may seep backward into the right atrium as a result of this (tricuspid valve regurgitation).

Each has a different position for the valve and degree of poor formation. Some folks have a valve that is only slightly off. Some have a valve that seriously leaks.

Other Cardiac Conditions Linked to the Ebstein Anomaly

Heart diseases that are frequently related include −

  • Hearts with holes. A hole between the two upper chambers of the heart called an atrial septal defect or an aperture called a patent foramen ovale is common in persons with Ebstein abnormality (PFO). All newborns have a PFO, which is a hole between the upper heart chambers that typically closes after birth. Some folks can continue to have it open without any problems.

  • These cracks might reduce the amount of oxygen in your blood, which would turn your face and lips blue (cyanosis).

  • Irregular heartbeats (arrhythmias). The heart's ability to function effectively is hampered by an irregular cardiac rhythm or fast heartbeats, particularly when the tricuspid valve is badly leaking. There are occasions when a highly rapid heartbeat results in fainting spells (syncope).

  • The syndrome of Wolff-Parkinson-White (WPW). WPW syndrome patients have an aberrant cardiac electrical circuit that can cause rapid heartbeats and fainting episodes.

Ebstein Anomaly: Symptoms

Ebstein anomalies with milder types may not show symptoms until later in adulthood. Some warning signs and symptoms include −

  • Breathing problems, especially after exertion

  • Fatigue

  • Irregular heart rhythms or palpitations in the heart (arrhythmias)

  • Reduced oxygen levels that result in blue discoloration of the lips and skin (cyanosis)

When to Visit a Doctor?

Speak to your doctor if you or your kid exhibits any heart failure signs or symptoms, such as feeling quickly tired or out of breath even during routine exercise, blue skin around the lips and nails, or swelling in your legs. He or she could suggest that you visit a physician who focuses on congenital heart disease (cardiologist).

Ebstein Anomaly: Risk Factors

While a baby's heart grows in the mother's pregnancy, congenital cardiac abnormalities like the Ebstein anomaly can happen.

What risk factors are connected to the issue is unclear to doctors. It is thought that both genetic and environmental factors are involved. A mother's usage of certain drugs, such as lithium, during pregnancy, or a family history of heart abnormalities may raise the likelihood of an Ebstein abnormality in the fetus.

Ebstein Anomaly: Diagnosis

The diagnosis of the Ebstein anomaly is mainly done based on history and some of the tests may be required for confirmation and to rule out underlying causes

  • Echocardiogram. This examination creates precise pictures of your heart using sound waves. It demonstrates the tricuspid valve's anatomy as well as how your heart's blood flows.

  • Electrocardiogram (ECG). The timing and length of your heartbeat are measured by sensors (electrodes) affixed to your chest and limbs. An ECG can help reveal issues with the rhythm and architecture of your heart.

  • Holter watch.

  • A chest X-ray.

  • Heart MRI. Using magnetic fields and radio waves, a cardiac MRI may provide precise pictures of your heart.

  • Oximetry through the pulse.

  • Stress test with exercise.

  • Study of electrophysiology (EP).

  • Catheterization of the heart. Using X-ray imaging, a long, thin tube (catheter) is placed into a blood artery in your groin, arm, or neck and directed to your heart.

Ebstein Anomaly: Treatment

The treatment is based on the severity of the symptoms. Your doctor may advise conservative or surgical treatment.

Conservative Treatment

Medication can help you manage your heart rate and have a normal heart rhythm if you have heart rhythm problems.

Also, your doctor could recommend meds including ones that stop water retention to treat the signs and symptoms of heart failure (diuretics).

Your doctor may recommend drugs to prevent blood clots if you have certain heart rhythm issues or a hole (atrial septal defect) between the upper heart chambers.

Surgical Treatment

Surgical treatment may be required in severe cases which include −

  • Fixing the tricuspid valve. For the valve to function correctly, surgeons enlarge the valve opening and narrow it.

  • Replacement of the tricuspid valve.

  • The atrial septal defect's closure.

  • Maze technique. Your surgeon may use the Maze method during valve repair or replacement surgery if you have a rapid heartbeat.

  • Ablation with a radiofrequency catheter. This operation could be carried out by your doctor if you have a rapid or irregular heartbeat. One or more catheters are inserted by your doctor into your blood veins and guided to your heart.

  • Transplanting of a heart. A heart transplant may be required if you have a significant Ebstein abnormality and poor heart health.

Ebstein Anomaly: Prevention

It may not be preventable because the precise etiology of the majority of congenital heart abnormalities is not understood. Genetic testing before or during pregnancy may be advised if you have a family history of congenital heart abnormalities or are at a high risk of being pregnant with a child who has one.


Most new-borns with Ebstein abnormality are discovered quite shortly after birth. As these newborns require NICU care, the ICU nurse must be knowledgeable about the treatment of congenital cardiac diseases.

While some babies have a single cardiac defect, others may also have involvement with other organ systems, necessitating the need for proper consultations. Hence, effective multidisciplinary cooperation between experts in congenital heart disease, cardiothoracic and pediatric surgeons, and intensivists is necessary for the therapy of Ebstein abnormality.

Dr. Durgesh Kumar Sinha
Dr. Durgesh Kumar Sinha


Updated on: 21-Apr-2023


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