Ebstein anomaly: Difference between revisions
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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>== | ||
[[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]] | ||
*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> | ||
* | |||
==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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*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, | **[[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== |
Latest revision as of 16:20, 10 April 2024
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
- History
- Physical Examination
Differential Diagnosis
- Other cyanotic congenital heart disease
Sick Neonate
THE MISFITS [12]
- Trauma
- Heart
- Congenital heart disease
- Hypovolemia
- Endocrine
- Metabolic
- Sodium
- Calcium
- Glucose
- Inborn errors of metabolism
- Seizure
- Formula / feeding problems
- Intestinal Disasters
- Toxin
- Sepsis
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
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
- Diuretics for heart failure [20]
- Digoxin [21]
- Procainamide, flecainide, propafenone, amiodarone
- 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]
- Recommended for: [22]
Disposition
- Cardiology or cardiothoracic surgery consultation
Complications[25]
- Cardiomegaly
- Hepatomegaly
- Congestive heart failure
- Cardiac dysrhythmias
- Thomboembolic events
See Also
External Links
Category
References
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ https://medlineplus.gov/ency/article/007321.htm
- ↑ https://medlineplus.gov/ency/article/007321.htm
- ↑ https://medlineplus.gov/ency/article/007321.htm
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ https://medlineplus.gov/ency/article/007321.htm
- ↑ https://medlineplus.gov/ency/article/007321.htm
- ↑ Brousseau T, Sharieff GQ. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am. 2006 Feb;53(1):69-84, vi.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ 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).
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ Attenhofer Jost, C., et al. (2007). Ebstein's anomaly. Circulation, 106: 277 - 285.
- ↑ https://medlineplus.gov/ency/article/007321.htm