Ebstein's anomaly
| Ebstein's anomaly | |
|---|---|
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| Pathological specimen and ultrasound image of a heart with Ebstein's anomaly: Abbreviations: RA: right atrium; ARV: atrialized right ventricle; FRV: fight ventricle; AL: anterior leaflet; SL: septal leaflet; LA: left atrium; LV: left ventricle; asterisk: grade II tethering of the tricuspid septal leaflet | |
| Classification and external resources | |
| Specialty | cardiology |
| ICD-10 | Q22.5 |
| ICD-9-CM | 746.2 |
| OMIM | 224700 |
| DiseasesDB | 4039 |
| MedlinePlus | 007321 |
| eMedicine | med/627 |
| MeSH | D004437 |
Ebstein's anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart.
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. A subsequent 'atrialization' of a portion of the morphologic right ventricle (which is then contiguous with the right atrium) is seen. This causes the right atrium to be large and the anatomic right ventricle to be small in size.
An enlargement of the aorta may occur; an increased risk of abnormality is seen in infants of women taking lithium during the first trimester of pregnancy (though some have questioned this) and in those with Wolff-Parkinson-White syndrome.
Ebstein's anomaly was named after Wilhelm Ebstein, who in 1866 described the heart of the 19-year-old Joseph Prescher.
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.
Typically, anatomic abnormalities of the tricuspid valve exist, with enlargement of the anterior leaflet of the valve. The other leaflets are described as being plastered to the endocardium.
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.
About 50% of individuals with Ebstein's anomaly have evidence of Wolff-Parkinson-White syndrome, secondary to the atrialized right ventricular tissue.
Other abnormalities that can be seen on the ECG include:
Ebstein's cardiophysiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred pharmacological treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers, and digoxin are contraindicated.
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