Current Management of Ebstein's Anomaly in the Adult

Ebstein’s anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. Minor anomalies of the tricuspid valve may not be recognized until adulthood whereas major anomalies leading to heart failure and cyanosis require surgical intervention earlier in life. Echocardiography is the imaging modality of choice for both diagnosis and management of patients with Ebstein’s anomaly. Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery. Pre-pregnancy evaluation should be considered in all Ebstein’s anomaly patients and for those who are cyanotic, surgical correction must be considered due to the maternal and fetal ramifications of cyanosis in pregnancy. Most acyanotic Ebstein’s anomaly patients are able to tolerate pregnancy with manageable or no complications. Those patients with mild anomalies and no right heart dilation can participate in sports whereas those with severe anomalies are discouraged from competitive sports. Physical activity as tolerated is important in all patients with adult congenital heart disease. Adult congenital heart specialists should evaluate patients prior to cardiac or noncardiac surgery. Longitudinal clinical follow-up in all Ebstein’s anomaly patients (both repaired and unrepaired) is warranted to follow for signs and symptoms of heart failure, arrhythmias, cyanosis, and other associated findings. This should be ideally performed in collaboration with an adult congenital heart center of excellence.