Difference between revisions of "Ebstein anomaly"

(Category)
 
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==Background==
 
==Background==
*Congenital cardiac abnormality
+
*[[congenital heart disease|Congenital cardiac abnormality]]
 
*Less than 1% of all congenital cardiac defects
 
*Less than 1% of all congenital cardiac defects
 
*Malformation of tricuspid valve and right ventricle <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
*Malformation of tricuspid valve and right ventricle <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
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==Clinical Features<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>==
 
==Clinical Features<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>==
 
*Cyanosis
 
*Cyanosis
*Right-sided heart failure
+
*Right-sided [[heart failure]]
*Dysrhythmias
+
*[[Dysrhythmias]]
 
*Sudden cardiac death
 
*Sudden cardiac death
 
==Differential Diagnosis==
 
*Other cyanotic congenital heart defects
 
 
==Evaluation==
 
  
 
*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<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>
 
**Widely and persistently S2<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Widely and persistently S2<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>
+
**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>
*Echo
+
 
 +
==Differential Diagnosis==
 +
*Other cyanotic [[congenital heart disease]]
 +
{{Sick neonate DDX}}
 +
 
 +
==Evaluation==
 +
*[[Echocardiography]]
 
**Apical displacement of septal leaflet of tricuspid valve
 
**Apical displacement of septal leaflet of tricuspid valve
 
**Tethering of tricuspid valve
 
**Tethering of tricuspid valve
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*Cardiac MR
 
*Cardiac MR
 
**Useful when echo image quality is inadequate<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Useful when echo image quality is inadequate<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
*ECG<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
+
*[[ECG]]<ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Tall, broad P waves due to right atrial enlargement
 
**Tall, broad P waves due to right atrial enlargement
 
**Complete or incompletely [[right bundle branch block]]
 
**Complete or incompletely [[right bundle branch block]]
 
**Small R wave in V1 and V2
 
**Small R wave in V1 and V2
 
**Bizarre QRS due to conduction abnormalities
 
**Bizarre QRS due to conduction abnormalities
*CXR
+
*[[CXR]]
 
**Enlarged cardiac silhouette <ref>https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132</ref>
 
**Enlarged cardiac silhouette <ref>https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132</ref>
 
**'Globe-shaped' heart with narrow waist <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**'Globe-shaped' heart with narrow waist <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
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**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
**Diuretics for heart failure <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
+
**[[Diuretics]] for heart failure <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
**Digoxin <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
+
**[[Digoxin]] <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
**Procainamide, flecanide, propafenone, amiodarone
+
**[[Procainamide]], [[flecainide]], propafenone, [[amiodarone]]
 
*Surgical repair
 
*Surgical repair
 
**Recommended for: <ref> 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).</ref>
 
**Recommended for: <ref> 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).</ref>
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***Significant cyanosis (resting SpO2 < 90%)
 
***Significant cyanosis (resting SpO2 < 90%)
 
***Severe symptomatic tricuspid regurgitation
 
***Severe symptomatic tricuspid regurgitation
***Transient ischemic attack or stroke
+
***[[Transient ischemic attack]] or [[stroke]]
 
**Tricuspid repair maintaining the native tricuspid valve whenever possible
 
**Tricuspid repair maintaining the native tricuspid valve whenever possible
 
**Catheter ablation for dysrhythmias <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
 
**Catheter ablation for dysrhythmias <ref>Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.</ref>
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==See Also==
 
==See Also==
 
+
*[[Congenital heart disease]]
  
 
==External Links==
 
==External Links==
  
 
==Category==
 
==Category==
[[Cardiology]]
+
[[Category:Cardiology]]
  
 
==References==
 
==References==
 
<references/>
 
<references/>

Latest revision as of 17:08, 17 August 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

Authors:

Claire