Ebstein's anomaly

Ebstein's anomaly

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 4039
ICD10 = ICD10|Q|22|5|q|20
ICD9 = ICD9|746.2
ICDO =
OMIM = 224700
MedlinePlus =
eMedicineSubj = med
eMedicineTopic = 627
MeshID = 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.

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. [WhoNamedIt|synd|435] [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.]

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

External links

* [http://heartcenter.seattlechildrens.org/conditions_treated/ebsteins_malformation.asp Ebstein's Malformation information] from Seattle Children's Hospital Heart Center
* [http://www.kumc.edu/instruction/medicine/pedcard/cardiology/pedcardio/ebsteinsdiagram.gifDiagram at kumc.edu]
* [http://www.mayoclinic.org/ebsteins-anomaly/abnormalities.html Overview and diagram] at Mayo Clinic
* [http://www.emedicine.com/med/topic627.htm Ebstein Anomaly article from eMedicine]


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