Ebstein Anomaly Recommendations for Ebstein Anomaly Referenced studies that support recommendations are summarized in Online Data Supplement 42. COR LOE Recommendations Diagnostic IIa B-NR In adults with Ebstein anomaly, CMR can be useful to determine anatomy, RV dimensions, and systolic function.S4.3.4-1,S4.3.4-2 IIa B-NR In adults with Ebstein anomaly, TEE can be useful for surgical planning if TTE images are inadequate to evaluate tricuspid valve morphology and function.S4.3.4-1 IIa B-NR Electrophysiological study with or without catheter ablation can be useful in the diagnostic evaluation of adults with Ebstein anomaly and ventricular preexcitation but without supraventricular IIa B-NR In adults with Ebstein anomaly, electrophysiological study (and catheter ablation, if needed) is reasonable before surgical intervention on the tricuspid valve even in the absence of preexcitation or Therapeutic I B-NR Surgical repair or reoperation for adults with Ebstein anomaly and significant TR is recommended when one or more of the following are present: HF symptoms, objective evidence of worsening exercise capacity, progressive RV systolic dysfunction by echocardiography or Therapeutic (Continued) I C-LD Catheter ablation is recommended for adults with Ebstein anomaly and high-risk pathway conduction or multiple accessory IIa B-NR Surgical repair or reoperation for adults with Ebstein anomaly and significant TR can be beneficial in the presence of progressive RV enlargement, systemic desaturation from right-to-left atrial shunt, paradoxical embolism, and/or atrial IIb B-NR Bidirectional superior cavopulmonary (Glenn) anastomosis at time of Ebstein anomaly repair may be considered for adults when severe RV dilation or severe RV systolic dysfunction is present, LV function is preserved, and left atrial pressure and LV end diastolic pressure are not Synopsis Ebstein anomaly is an uncommon congenital heart defect occurring in about 0.005% of live births.S4.3.4-16 It is a malformation of the tricuspid valve and the right ventricle and varies in severity, including babies who do not survive infancy, asymptomatic adults diagnosed incidentally in the sixth and seventh decades of life, and many variations in severity between those extremes. 