Ebstein Anomaly Workup

Updated: Jan 17, 2017
  • Author: Kamran Riaz, MD; Chief Editor: Yasmine Subhi Ali, MD, FACC, FACP, MSCI  more...
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Workup

Imaging of Ebstein Anomaly

Chest radiography

Chest radiographs may reveal the following in patients with Ebstein anomaly:

  • Normal findings
  • Cardiomegaly
  • Small aortic root and main pulmonary artery shadow
  • Decreased pulmonary vasculature
  • Large right atrium

Echocardiography

Echocardiogram is the criterion standard for diagnosis.

M-mode echocardiography

  • Paradoxical septal motion
  • Dilated right ventricle
  • Delayed closure of tricuspid valve leaflets more than 65 milliseconds after mitral valve closure

Two-dimensional echocardiography

  • Apical displacement of the septal leaflet of tricuspid leaflet of greater than 8 mm/m 2: Most specific sign
  • Abnormalities in morphology and septal attachment of the septal and anterior tricuspid leaflets
  • Eccentric leaflet coaptation
  • Dilated right atrium
  • Dilated right ventricle with decreased contractile performance
  • Various left heart structural abnormalities (described recently in up to 39% of patients with Ebstein abnormality)

​Doppler studies

  • Varying degrees of tricuspid regurgitation
  • Excludes associated shunts

Assessment of severity and surgical options by echocardiography

  • Functional right ventricular area less than 35% of total right ventricular area or an atrialized to functional right ventricular ratio greater than 0.5 associated with unfavorable prognosis
  • Functional right ventricular size
  • Degree of septal leaflet displacement
  • Amount of leaflet tethering
  • Magnitude of leaflet deformity and dysplasia
  • Aneurysmal dilatation of right ventricular outflow tract (right ventricular outflow tract-to-aortic root ratio of >2:1 on parasternal short axis view)
  • Moderate-to-severe tricuspid regurgitation

Magnetic resonance imaging (MRI)

Cine MRI is currently not used routinely; it may become a useful noninvasive modality in the future. Right/Left heart volume index correlates well with heart failure markers. [9]

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Electrocardiography

Twelve-lead electrocardiographic (ECG) features of patients with Ebstein anomaly include the following:

  • Rhythm is usually normal sinus; intermittently, supraventricular tachycardia (SVT), paroxysmal SVT, atrial flutter, atrial fibrillation, or ventricular tachycardia may occur
  • Abnormal P waves are consistent with right atrial enlargement
  • PR interval is most commonly prolonged; it may be normal or short in patients with Wolff-Parkinson-White (WPW) syndrome
  • QRS complex may demonstrate right bundle branch block (BBB); it may also be low voltage in many patients
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Other Procedures

Cardiac catheterization

Currently, cardiac catherization is rarely performed for the evaluation of Ebstein anomaly. It can confirm echocardiographic findings: Cardiac catherization can reveal right ventricular electrical activity on the intracardiac electrocardiogram (ECG) with simultaneous right atrial pressure and waveform when the catheter is withdrawn from the right ventricle, back across the tricuspid valve into the right atrium.

Electrophysiologic studies

Of patients with Ebstein anomaly, 25-30% have accessory pathways, and 5-25% have evidence of preexcitation on the surface ECG. Electrophysiologic (EP) studies in patients with tachyarrhythmias can delineate accessory conduction pathways and guide ablative therapy. Right-sided pathways are more common, and 50% of the patients have multiple pathways.

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