Emergency: requires immediate attention
Ebstein anomaly
Alerts and Notices
Synopsis
Clinical features are variable based on the degree of tricuspid valve impairment. While mild cases may appear asymptomatic, patients with mild to moderate impairment may present with exertional dyspnea, fatigue, heart murmur, and palpitations. Severe anomalies may present at birth with cyanosis and cardiac failure. Atrial tachyarrhythmias are not infrequently seen and may originate from accessory pathways.
Ebstein anomaly is often associated with other structural heart diseases such as secundum-type atrial septal defect, ventricular septal defect, pulmonary outflow obstruction, patent ductus arteriosus, mitral valve prolapse, bicuspid aortic valve, and accessory conduction pathways (25% of cases).
Maternal exposure to benzodiazepines and lithium therapy can predispose the neonate to this condition.
Codes
ICD10CM:Q22.5 – Ebstein's anomaly
SNOMEDCT:
204357006 – Ebstein's anomaly of tricuspid valve
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Tricuspid regurgitation
- Uhl anomaly
- Mitral regurgitation – murmur is more in expiration
- Aortic stenosis – crescendo-decrescendo murmur
- Atrial septal defect
- Tricuspid atresia
- Transposition of the great vessels
- Carcinoid heart disease (see carcinoid syndrome)
- Arrhythmogenic right ventricular cardiomyopathy
- Wolff-Parkinson-White syndrome
- Traumatic injury of tricuspid valve – instrumentation history
Best Tests
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Management Pearls
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Therapy
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References
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Last Reviewed:10/03/2018
Last Updated:01/13/2022
Last Updated:01/13/2022
Emergency: requires immediate attention
Ebstein anomaly