Ebstein Anomaly

Ebstein's anomaly is a congenital heart defect that involves an abnormally developed tricuspid valve. Learn more about its effects on the heart physiology in today's episode.

This episode was written by pediatricians Tammy Yau and Lidia Park with content support from Daniel Cortez (pediatric cardiology). Pediatricians Tammy and Lidia take full responsibility for any errors or misinformation.

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Key Points:

• Ebstein's anomaly is characterized by an abnormally placed tricuspid valve causing atrialization of the right ventricle. This can lead to single ventricle physiology.
• Wolff-Parkinson-White (WPW), right bundle branch blocks (RBBB), mitral valve prolapse, mitral valve dysplasia, bicuspid aortic valve, ventricular septal defect, and coarctation of the aorta can all be associated with Ebstein's anomaly
• Treatment of Ebstein's anomaly requires a patient to be stable enough to tolerate surgical correction like aortopulmonary shunting, PDA stenting, tricuspid valvuloplasty, and/or single ventricle palliation. Some patients will require inhaled nitrous oxide, Prostaglandin E, antiarrhythmics, and/or ECMO as well

Sources:

• Truong et al. “Ebstein Anomaly in the Fetus: Preparing for the Perfect Storm”.
• Neoreviews (2011) 12 (5): e260–e269. https://doi.org/10.1542/neo.12-5-e260