Preoperative Physiology, Imaging, and Management of Ebstein's Anomaly of the Tricuspid Valve.

Ebstein's anomaly of the tricuspid valve (TV) refers to an embryological derangement of TV formation causing tethering of the septal and posterior leaflets of the valve to the underlying myocardium and apical displacement of the effective valve annulus, resulting in significant TV insufficiency and dilation of the right heart structures. The pathological abnormalities of the valve can vary significantly, resulting in a wide range of clinical presentations. Fetal diagnosis and neonatal presentations of the disease are typically the most severe and are associated with the highest mortality rates. Patients with less-severe disease will present later in life with symptoms of right heart failure and tachyarrhythmias. Medical and surgical management strategies are driven by the age at presentation, severity of disease, and any associated cardiac abnormalities. There are an increasing number of surgical options focused on valve repair.