Ebstein anomaly: Difference between revisions
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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== | ||
| Line 53: | Line 53: | ||
*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 | ||
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]
- 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