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Ebstein Anomaly

Practice Essentials

Ebstein anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet.

Signs and symptoms

Patients can have a variety of symptoms related to the anatomic abnormalities of Ebstein anomaly and their hemodynamic effects or associated structural and conduction system disease, including the following:

Cyanosis

Fatigue and dyspnea

Palpitations and sudden cardiac death

Symptoms of right heart failure, such as edema and ascites

Other less common presenting symptoms include the following:

Brain abscess due to right-to-left shunt

Bacterial endocarditis

Paradoxical embolism, stroke, and transient ischemic attacks

See Clinical Presentation for more detail.

Diagnosis

Physical findings in patients with Ebstein anomaly span a spectrum from subtle to dramatic. They may include the following:

Cyanosis and clubbing

Precordial asymmetry

Jugular venous pulse

Arterial pulses: Usually normal but are diminished late in the disease course

Heart sounds: Widely split first heart sound with loud tricuspid component and soft/absent mitral component in the presence of prolonged PR interval; usually normal second heart sound but may be widely split when pulmonary component delayed due to right bundle-branch block; third and fourth heart sounds commonly present, even in the absence of congestive heart failure

Testing

A 12-lead electrocardiogram may demonstrate the following findings in patients with Ebstein anomaly:

Normal sinus rhythm

Intermittent/paroxysmal supraventricular tachycardia, atrial flutter/fibrillation, ventricular tachycardia

Abnormal P waves consistent with right atrial enlargement

Prolonged PR interval; may be normal/short in patients with Wolff-Parkinson-White syndrome

Right bundle branch block and/or low voltage QRS complex

Imaging studies

The following imaging studies may be used to assess patients with suspected Ebstein anomaly:

Chest radiography

Echocardiography: Includes M- mode, 2-dimensional, and Doppler studies

Cine magnetic resonance imaging: In selected patients; not used routinely

See Workup for more detail.

Management

Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Treatment options include medical therapy, radiofrequency ablation, and surgical therapy.

Pharmacotherapy

Ebstein anomaly requires drug treatment for cardiovascular consequences resulting from tricuspid atrialization of the right ventricle, valvular regurgitation, and septal defects. Patients may require antibiotic prophylaxis for bacterial endocarditis.

The following medications are used in patients with Ebstein anomaly:

Diuretics (eg, furosemide)

Cardiac glycosides (eg, digoxin)

Angiotensin-converting enzyme inhibitors (eg, enalapril)

Nonpharmacotherapy

Radiofrequency ablation of the accessory pathways is an alternative to medication for treatment of arrhythmias. Curative therapy of supraventricular tachycardia with radiofrequency ablation is the treatment of choice. However, in patients without significant structural heart disease, the success rate of this procedure is lower.

Surgical option

Surgical intervention includes the following:

Correction of the underlying tricuspid valve and right ventricular abnormalities

Correction of any associated intracardiac defects

Palliative procedures in early days of life as a bridge to more definitive surgical treatment later

Surgical treatment of associated arrhythmias (eg, ablation of the accessory pathways; maze procedure for atrial arrhythmias)

Cardiac transplantation in selected patients

See Treatment and Medication for more detail.

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