Ebstein anomaly

Background

Ebstein anomaly showing the downward displacement of the tricuspid valve from its normal position in the fibrous ring down into the right ventricle.
  • Congenital cardiac abnormality
  • Less than 1% of all congenital cardiac defects
  • Malformation of tricuspid valve and right ventricle [1]
    • Septal and posterior leaflets of tricuspid valve adhere to underlying myocardium
    • Functional annulus of tricuspid valve displaced toward right ventricular apex
    • Portion of the right ventricle is 'atrialized' with dilation and hypertrophy or thinning of the wall
    • Anterior leaflet of tricuspid valve may be redundant or tethered
    • Right AV junction dilated
    • Right-to-left cardiac shunt

Clinical Features[2]

An example of clubbing.

Differential Diagnosis

Congenital Heart Disease Types

Sick Neonate

THE MISFITS [13]

Evaluation

ECG of a woman with Ebstein's anomaly showing 1) signs of right atrial enlargement (best seen in V1), 2) broad and tall P waves ("Himalayan" P waves), and 3) right bundle-branch block pattern and a first-degree atrioventricular block (prolonged PR-interval) due to intra-atrial conduction delay. No evidence of a Kent-bundle is seen in this patient. The T wave inversion in V1-4 and a marked Q wave in III occur; these changes are characteristic for Ebstein's anomaly and do not reflect ischemic ECG changes in this patient.
Comparison of echo to pathological specimen in a heart with Ebstein's anomaly. Asterix shows grade II tethering of the tricuspid septal leaflet. Other abbreviations: right atrium (RA), atrialized right ventricle (AVR), functional right ventricle (FRV), anterior leaflet (AL), septal leaflet (SL), left atrium (LA), and left ventricle (LV).
  • Echocardiography
    • Apical displacement of septal leaflet of tricuspid valve
    • Tethering of tricuspid valve
    • Right atrial enlargement
    • Atrialization of right ventricle
    • Tricuspid regurgitation
  • Cardiac MR
    • Useful when echo image quality is inadequate[14]
  • ECG[15]
    • Tall, broad P waves due to right atrial enlargement
    • Complete or incompletely right bundle branch block
    • Small R wave in V1 and V2
    • Bizarre QRS due to conduction abnormalities
  • CXR
    • Enlarged cardiac silhouette [16]
    • 'Globe-shaped' heart with narrow waist [17]

Management

  • Supportive care
    • Asymptomatic patients with no right to left shunting and minimal cardiomegaly may only require regular monitoring[18][19]
    • Endocarditis prophylaxis
    • Avoidance of sport in moderate or severe disease (patients with mild disease may participate in sport)[20]
  • Medical management
  • Surgical repair
    • Recommended for: [23]
      • Limited exercise capacity (NYHA III - IV)
      • Increasing heart size (cardiothoracic ration > 0.65)
      • Significant cyanosis (resting SpO2 < 90%)
      • Severe symptomatic tricuspid regurgitation
      • Transient ischemic attack or stroke
    • Tricuspid repair maintaining the native tricuspid valve whenever possible
    • Catheter ablation for dysrhythmias [24]
    • Pacemaker insertion for persistent dysrhythmias [25]

Disposition

  • Cardiology or cardiothoracic surgery consultation

Complications[26]

  • Cardiomegaly
  • Hepatomegaly
  • Congestive heart failure
  • Cardiac dysrhythmias
  • Thomboembolic events

See Also

External Links

Category

References

  1. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  2. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  3. ↑ https://medlineplus.gov/ency/article/007321.htm
  4. ↑ https://medlineplus.gov/ency/article/007321.htm
  5. ↑ https://medlineplus.gov/ency/article/007321.htm
  6. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  7. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  8. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  9. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  10. ↑ https://medlineplus.gov/ency/article/007321.htm
  11. ↑ https://medlineplus.gov/ency/article/007321.htm
  12. ↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease
  13. ↑ Brousseau T, Sharieff GQ. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am. 2006 Feb;53(1):69-84, vi.
  14. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  15. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  16. ↑ https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132
  17. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  18. ↑ https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132
  19. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  20. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  21. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  22. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  23. ↑ Silversides, C, et al. (2010). Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Outflow tract obstruction, coarctation of the aorta, tetralogy or Fallot, Ebstein anomaly, and Marfan's syndrome. Canadian Journal of Cardiology; 26(3).
  24. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  25. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  26. ↑ https://medlineplus.gov/ency/article/007321.htm
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