Ebstein Anomaly of the Tricuspid Valve

Ebstein anomaly is rare and presents a spectrum of anatomic and functional severity. Anatomic severity depends on the degree of displacement of the anterior and posterior leaflets of the tricuspid valve into the right ventricle and the size of the resultant right ventricular chamber. Tricuspid regurgitation results. Functional severity is affected by the degree of regurgitation, magnitude of atrial right-to-left shunt, degree of right atrial and ventricular sizes, and ventricular function. Pre-excitation coexists in some patients. Neonates can present in severe distress and with intense cyanosis; they are a therapeutic challenge and have a high mortality. Indications for operation in older patients include cardiac size, ventricular function, and symptoms. A number of techniques have been described to repair the valve and at the time of the operation the atrial communication is also closed.