Ebstein anomaly

The printable version is no longer supported and may have rendering errors. Please update your browser bookmarks and please use the default browser print function instead.

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]

  • Cyanosis
  • Right-sided heart failure
  • Dysrhythmias
  • Sudden cardiac death

Differential Diagnosis

  • Other cyanotic congenital heart defects

Evaluation

  • History
  • Physical Examination
    • Jugular venous V wave[6]
    • Widely and persistently S2[7]
    • Systolic murmur[8]
    • Digital clubbing[9]
    • Tachypnea [10]
    • Tachycardia [11]
  • Echo
    • 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[12]
  • ECG[13]
    • 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 [14]
    • 'Globe-shaped' heart with narrow waist [15]

Management

  • Supportive care
    • Asymptomatic patients with no right to left shunting and minimal cardiomegaly may only require regular monitoring[16][17]
    • Endocarditis prophylaxis
    • Avoidance of sport in moderate or severe disease (patients with mild disease may participate in sport)[18]
  • Medical management
    • Diuretics for heart failure [19]
    • Digoxin [20]
    • Procainamide, flecanide, propafenone, amiodarone
  • Surgical repair
    • Recommended for: [21]
      • 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 [22]
    • Pacemaker insertion for persistent dysrhythmias [23]

Disposition

  • Cardiology or cardiothoracic surgery consultation

Complications[24]

  • 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. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  13. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  14. ↑ https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132
  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. ↑ 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. ↑ 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).
  22. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  23. ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
  24. ↑ https://medlineplus.gov/ency/article/007321.htm
Retrieved from "https://www.wikem.org/w/index.php?title=Ebstein_anomaly&oldid=209278"