Ebstein's Anomaly of the Tricuspid Valve with Associated Mitral Valve Prolapse

Ebstein's anomaly is primarily a malformation of the tricuspid valve. However, mitral valve abnormalities, including mitral valve prolapse, have been reported in Ebstein's anomaly patients. ^1,2 The mitral valve prolapse may progress with time and result in substantial hemodynamic changes that worsen the patient's condition.

A 27-year-old woman was evaluated for severe progressive shortness of breath. Quadruple heart sounds, a systolic click, and a holosystolic murmur were audible at the cardiac apex. Transthoracic echocardiography was limited technically because of the patient's obesity. Transesophageal echocardiography was performed, which revealed Ebstein's anomaly of the tricuspid valve with associated mitral valve prolapse. The tricuspid valve was displaced away from the atrioventricular junction into the right ventricular cavity. The inflow portion of the right ventricle was incorporated into the right atrium, resulting in an enlarged right atrium and a smaller right ventricle. The anterior and posterior cusps of the mitral valve prolapsed into the left atrium during systole (Fig. 1). Both the tricuspid and the mitral valve were severely regurgitant, which contributed to the patient's excessive shortness of breath.

Fig. 1 Transesophageal echocardiogram (4-chamber view) during systole, revealing downward displacement of the septal and posterior cusps of the tricuspid valve (TV) into the right ventricular (RV) cavity; incorporation of the inflow portion of the right ventricle into the right atrium; an enlarged right atrium (RA); a smaller right ventricle; and prolapse of the anterior and posterior cusps of the mitral valve (MV) into the left atrium (LA).

LV = left ventricle