Difference between revisions of "Ebstein anomaly"

 
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*Right-sided [[heart failure]]
 
*Right-sided [[heart failure]]
 
*[[Dysrhythmias]]
 
*[[Dysrhythmias]]
*Sudden cardiac death
+
*Sudden [[cardiac arrest|cardiac death]]
  
 
*History
 
*History
 
**[[Cough]] <ref>https://medlineplus.gov/ency/article/007321.htm</ref>
 
**[[Cough]] <ref>https://medlineplus.gov/ency/article/007321.htm</ref>
 
**[[Failure to thrive (peds)|Failure to thrive]]<ref>https://medlineplus.gov/ency/article/007321.htm</ref>
 
**[[Failure to thrive (peds)|Failure to thrive]]<ref>https://medlineplus.gov/ency/article/007321.htm</ref>
**Fatigue<ref>https://medlineplus.gov/ency/article/007321.htm</ref>
+
**[[Fatigue]]<ref>https://medlineplus.gov/ency/article/007321.htm</ref>
 
*Physical Examination
 
*Physical Examination
 
**Jugular venous V wave<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Jugular venous V wave<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
Line 25: Line 25:
 
**Systolic [[murmur]]<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Systolic [[murmur]]<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Digital clubbing<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Digital clubbing<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
**Tachypnea <ref>https://medlineplus.gov/ency/article/007321.htm</ref>
+
**[[Tachypnea]] <ref>https://medlineplus.gov/ency/article/007321.htm</ref>
**Tachycardia <ref>https://medlineplus.gov/ency/article/007321.htm</ref>
+
**[[Tachycardia]] <ref>https://medlineplus.gov/ency/article/007321.htm</ref>
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
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*Supportive care
 
*Supportive care
 
**Asymptomatic patients with no right to left shunting and minimal cardiomegaly may only require regular monitoring<ref>https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132</ref><ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Asymptomatic patients with no right to left shunting and minimal cardiomegaly may only require regular monitoring<ref>https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132</ref><ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
**Endocarditis prophylaxis
+
**[[Endocarditis]] prophylaxis
 
**Avoidance of sport in moderate or severe disease (patients with mild disease may participate in sport)<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Avoidance of sport in moderate or severe disease (patients with mild disease may participate in sport)<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
*Medical management
 
*Medical management

Latest revision as of 18:53, 24 September 2019

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

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. 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