Pediatric and Congenital Heart Disease Session 5: Does CMR Impact the Management of Ebstein's Anomaly?-Approach to the Adult with Ebstein Anomaly: Imaging for Complex Decision Making

Approach to the Adult with Ebstein Anomaly: Imaging for Complex Decision Making

Video Transcription

Video Summary

The speaker discusses Ebstein anomaly in adults, emphasizing its extreme variability in anatomy, physiology, symptoms, and age at presentation. Because no single severity measure exists, management relies on imaging and clinical data to guide timing and type of intervention. MRI is highlighted as especially useful for assessing valve morphology, right ventricular size and function, regurgitant fraction, forward flow, left ventricular filling, and associated abnormalities such as mitral valve prolapse or noncompaction. It also helps evaluate outcomes after procedures like cone repair or cavo-pulmonary shunts. A simple MRI marker, the “Ebstein rotation angle,” may predict suitability and success of cone repair, especially in children, and risk of dehiscence in adults. However, MRI has limitations, including poor temporal resolution, arrhythmia-related image quality issues, and difficulty predicting myocardial behavior after surgery. The speaker concludes that multidisciplinary, longitudinal, multi-institutional data are needed to improve decision-making and outcome prediction.

Keywords

Ebstein anomaly

MRI assessment

cone repair

right ventricular function

multidisciplinary management