Ebstein anomaly

Ebstein’s anomaly is a rare congenital heart disorder occurring in ≈1 per 200 000 live births and accounting for <1% of all cases of congenital heart disease.^2–6 This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation.”^7,8 The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly.^7,8 At autopsy, Ebstein described an enlarged and fenestrated anterior leaflet of the tricuspid valve. The posterior and septal leaflets were hypoplastic, thickened, and adherent to the right ventricle. There was also a thinned and dilated atrialized portion of the right ventricle, an enlarged right atrium, and a patent foramen ovale⁹ (Figure 1). By 1950, only 3 cases of this anomaly had been published.