Ebstein anomaly

Background
  • 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]

Differential Diagnosis

Sick Neonate

THE MISFITS [12]

Evaluation
  • 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[13]
  • ECG[14]
    • 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 [15]
    • 'Globe-shaped' heart with narrow waist [16]

Management
  • Supportive care
    • Asymptomatic patients with no right to left shunting and minimal cardiomegaly may only require regular monitoring[17][18]
    • Endocarditis prophylaxis
    • Avoidance of sport in moderate or severe disease (patients with mild disease may participate in sport)[19]
  • Medical management
  • Surgical repair
    • Recommended for: [22]
      • 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 [23]
    • Pacemaker insertion for persistent dysrhythmias [24]

Disposition
  • Cardiology or cardiothoracic surgery consultation

Complications[25]
  • Cardiomegaly
  • Hepatomegaly
  • Congestive heart failure
  • Cardiac dysrhythmias
  • Thomboembolic events

See Also

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. Brousseau T, Sharieff GQ. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am. 2006 Feb;53(1):69-84, vi.
  13. Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  14. Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  15. https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132
  16. Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  17. https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132
  18. Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  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. 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).
  23. Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  24. Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  25. https://medlineplus.gov/ency/article/007321.htm
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