Ebstein anomaly

Abstract

Ebstein anomaly is a relatively rare lesion, which represents less than 1 % of all cases of congenital heart disease and has a prevalence of about 3-5 per 100,000 live births. Although Ebstein anomaly primarily relates to a malformation of the tricuspid valve with consequent effects on right ventricular development, there is, in fact, a wide variation of abnormalities and associated lesions which may have significant impact on disease prognosis. Ebstein anomaly is unique in that there is a broad spectrum of disease, varying from the severe neonatal form which continues to carry a dismal prognosis, even in contemporary series, to the milder form which may only become manifest in adult life, if at all.