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Emergency: requires immediate attention
Ebstein anomaly
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Emergency: requires immediate attention

Ebstein anomaly

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Contributors: Nishit Biniwale MD, Bipul Baibhav MD, FACC, FASE
Other Resources UpToDate PubMed

Synopsis

Ebstein anomaly is a rare, complex congenital cardiac malformation characterized by apical displacement of the tricuspid valve leaflets due to failed delamination and concomitant fibrous attachments to the right ventricular endocardium, leading to "atrialization" of the right ventricle. It has a heterogeneous genetic predisposition and is reported in 1-5 of every 200 000 live births with no gender predilection. It accounts for about 1% of all congenital cardiac abnormalities.

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.

For more information, see OMIM.

Codes

ICD10CM:
Q22.5 – Ebstein's anomaly

SNOMEDCT:
204357006 – Ebstein's anomaly of tricuspid valve

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Last Reviewed: 10/04/2018
Last Updated: 10/04/2018
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Emergency: requires immediate attention
Ebstein anomaly
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Ebstein anomaly (Infants and Adolescents) : Cough, Fatigue, Cardiomegaly, Cyanosis, Exertional dyspnea, Failure to thrive, Heart murmur, Arrhythmia, Tachypnea
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