A rare tricuspid anomaly

Ebstein anomaly is an uncommon form of congenital heart disease, primarily of the tricuspid valve. It is usually diagnosed by observing displacement of the annular attachment of the septal, and often mural, leaflets of the tricuspid valve into the right ventricle with dysplasia of the valvular leaflets, although there are multiple variations with a spectrum of pathologic features. Cross-sectional echocardiography is the method of choice for the diagnosis of this rare cardiac malformation. Most patients with this condition also have an abnormal chest radiograph with globular cardiomegaly resulting from right atrial enlargement. Associated cardiac anomalies are present in many patients, with atrial septal defect being the most common. It is important to recognize the pathologic features of this disorder because they might influence the approach to surgical treatment, clinical course, and prognosis. The method of surgical treatment of patients who require surgery remains controversial.