Heart rupture and tamponade with a short review of cause of death in Ebstein anomaly.

摘要

Ebstein anomaly (EA), as first described by Wilhelm Ebstein in 1866, is not an unusually rare heart malformation and certainly is one of the most common affecting the tricuspid valve. The anomaly is caused by an incomplete embryologic process of undermining the free right ventricular wall and a failed resorption of the apical portion of the valve tissue. This leads to a displacement of the tricuspid leaflets closer to the apex, while the right atrium becomes enlarged (1,2). The right atrium is enlarged due to two main reasons: (i) atrialization of the right ventricular wall, which remains above the tricuspid valve; and (ii) hemodynamic issues of valve insufficiency and regurgitation. EA is usually associated with other heart malformations such as septal defects, other valvular malformations, and defects of the conduction system, as in Wolff-Parkinson-White syndrome (WPW) (3).