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Ebstein's anomaly (EA) is a rare and complex congenital heart disease (CHD) of the tricuspid valve (TV), in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart. EA was named after Wilhelm Ebstein, who in 1866 described the heart of the 19 year old Joseph Prescher. It occurs in about 1 per 200 live births, and accounting for less than 1% of all CHD. EA may present at any age with a variety of hemodynamic and electrophysiologic sequel. The EA can coexist with others CHD, genetic syndromes, or being associated with Wolff-Parkinson-White (WPW) syndrome. Surgical management of EA depends of the patient age, the clinical presentation and the association with other CHD. Cardiothoracic ratio upper to 0.60 is a factor of poor prognostic in patients with Ebstein’s anomaly and the prognostic is worse if is associated with others congenital heart disease.
Source: Ebstein’s Anomaly: An Impressive Cardiothoracic Ratio
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