Ebstein anomaly: Difference between revisions
 
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==Background==
==Background==
*Congenital cardiac abnormality
[[File:EbsteinAnomaly.svg|thumb|Ebstein anomaly showing the downward displacement of the tricuspid valve from its normal position in the fibrous ring down into the right ventricle.]]
*[[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>==
[[File:ClubbingFingers1.jpg|thumb|An example of [[clubbing]].]]
*Cyanosis
*Cyanosis
*Right-sided heart failure
*Right-sided [[heart failure]]
*Dysrhythmias
*[[Dysrhythmias]]
*Sudden cardiac death
*Sudden [[cardiac arrest|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==
{{Congenital heart disease DDX}}
{{Sick neonate DDX}}
 
==Evaluation==
[[File:Ebstein's anomaly ECG.png|thumb|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.]]
[[File:Ebstein4.jpg|thumb|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
**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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*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
**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 17:58, 6 November 2024

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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