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| Ebstein's anomaly Classification and external resources |
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| ICD-10 | Q22.5 |
|---|---|
| ICD-9 | 746.2 |
| OMIM | 224700 |
| DiseasesDB | 4039 |
| eMedicine | med/627 |
| MeSH | D004437 |
Ebstein's anomaly is a congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart.
Contents |
Presentation
The annulus of the valve is still in the normal position. The valve leaflets, however, are to a varying degree, attached to the walls and septum of the right ventricle. There is subsequent atrialization of a portion of the morphologic right ventricle (which is then contiguous with the right atrium). This causes the right atrium to be large and the anatomic right ventricle to be small in size.
Risk factors
There may be an increased risk of this abnormality in infants of women taking lithium during the first trimester of pregnancy, and in those with Wolff-Parkinson-White syndrome.
History
Ebstein's anomaly was named after Wilhelm Ebstein.[1][2]
Related abnormalities
While Ebstein's anomaly is defined as the congenital displacement of the tricuspid valve towards the apex of the right ventricle, it is often associated with other abnormalities.
Anatomic abnormalities
Typically, there are anatomic abnormalities of the tricuspid valve, with enlargement of the anterior leaflet of the valve.
About 50% of individuals with Ebstein's anomaly have an associated shunt between the right and left atria, either an atrial septal defect or a patent foramen ovale.
Electrophysiologic abnormalities
About 50% of individuals with Ebstein's anomaly have evidence of Wolff-Parkinson-White syndrome, secondary to the atrialized right ventricular tissue.
Management
Pharmacological
Ebstein's cardiophysiology typically presents as an atrioventricular reentrant tachycardia with associated preexcitation. In the acute setting with EKG evidence of preexcitation, the preferred pharmacological treatment agents include procainamide or ibutilide since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion.
References
- ^ synd/435 at Who Named It
- ^ W. Ebstein. Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Archiv für Anatomie, Physiologie und wissenschaftliche Medicin, Leipzig, 1866, 238-254.
External links
- Ebstein's Malformation information from Seattle Children's Hospital Heart Center
- Diagram at kumc.edu
- Overview and diagram at Mayo Clinic
- Ebstein Anomaly article from eMedicine
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Wikipedia content modification information:
- This page was last modified on 2 September 2008, at 22:45.
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