Ebstein Anomaly Workup

  • Author: Kamran Riaz, MD; Chief Editor: Park W Willis IV, MD   more...
 
Updated: May 31, 2011
 

Imaging Studies

  • Chest radiographs can reveal the following:
    • Normal findings
    • Cardiomegaly
    • Small aortic root and main pulmonary artery shadow
    • Decreased pulmonary vasculature
    • Large right atrium
  • Echocardiogram is the criterion standard for diagnosis.
    • M-mode
      • Paradoxical septal motion
      • Dilated right ventricle
      • Delayed closure of tricuspid valve leaflets more than 65 milliseconds after mitral valve closure
    • Two-dimensional
      • Apical displacement of the septal leaflet of tricuspid leaflet of greater than 8 mm/m2 - 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
  • Cine MRI is currently not used routinely; it may become a useful noninvasive modality in the future.
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Other Tests

Twelve-lead ECG

  • Rhythm
    • Usually normal sinus findings
    • Intermittently SVT, paroxysmal SVT, atrial flutter, atrial fibrillation, ventricular tachycardia
  • Abnormal P waves consistent with right atrial enlargement
  • PR interval
    • Most commonly prolonged
    • May be normal or short in patients with WPW syndrome
  • QRS complex
    • RBBB
    • Low voltage in many patients
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Procedures

  • Cardiac catheterization
    • Rarely performed today
    • Confirms echocardiographic findings
    • Can reveal right ventricular electrical activity on the intracardiac 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.
    • Fifty percent of the patients have multiple pathways.
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Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

Kamran Riaz, MD  Clinical Assistant Professor, Department of Internal Medicine, Section of Cardiology, Wright State University School of Medicine

Kamran Riaz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Society of Echocardiography, Ohio State Medical Association, and Royal College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association

Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

References

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