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Ebstein's Anomaly

A rare congenital heart disease in which the septal and posterior tricuspid leaflets are displaced into the right ventricle.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

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What is Ebstein's Anomaly?

Ebstein's anomaly is characterized by apical displacement of the septal and posterior leaflets of the tricuspid valve into the right ventricle. The right atrium becomes enlarged, while the functional right ventricle is reduced.

A significant proportion of cases are associated with an atrial septal defect, patent foramen ovale, and Wolff–Parkinson–White (WPW) syndrome. The severity spectrum ranges from asymptomatic adults to severe neonatal cyanosis.

Treatment is planned according to the severity of symptoms and valve insufficiency. Mild cases are observed, while advanced cases may require valve repair or replacement.

Symptoms

Cyanosis and heart failure in the newborn
Palpitations and episodes of supraventricular tachycardia
Exertional dyspnea and fatigue
Signs of right heart failure (hepatomegaly, peripheral edema)
Atrial arrhythmias (particularly atrial fibrillation)
Tricuspid regurgitation murmur
Paradoxical embolism and stroke risk

Risk Factors

Lithium use in pregnancy
Family history of congenital heart disease
Coexisting WPW syndrome
Benzodiazepine exposure in pregnancy
Genetic syndromes
Viral infections in pregnancy
Family history of arrhythmia

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • In the newborn with cyanosis and respiratory distress
  • Recurrent palpitations and syncopal episodes
  • Before planning pregnancy
  • Stroke symptoms suggestive of paradoxical embolism

Treatment Methods

01
Annual echocardiographic follow-up in mild cases
02
Antiarrhythmic therapy or ablation for arrhythmias
03
Diuretics and ACE inhibitors for heart failure
04
Surgery: tricuspid repair (cone reconstruction) or replacement
05
Atrial septal defect closure
06
Anticoagulant therapy (for paradoxical embolism or AF)

Which Department to Visit?

You can visit our Kardiyoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.