Ebstein’s Anomaly
from OrphaChat — a Rare Disease Podcast Hosted by RCH
These sources detail the clinical management and pathophysiological characteristics of adults with congenital heart disease, with a primary focus on Ebstein’s anomaly. This specific malformation involves the displacement of the tricuspid valve, leading to heart failure, cyanosis, and a high prevalence of arrhythmias such as Wolff-Parkinson-White syndrome. Diagnostic strategies include multimodal imaging and specialized scoring systems to predict mortality across different life stages. Treatment options range from pharmacological interventions to complex surgical techniques like the cone repair, which seeks to reconstruct a functional valve. Modern guidelines advocate for lifelong multidisciplinary care at dedicated centres to address long-term complications and improve survival. Comprehensive resources also highlight the importance of expert anaesthesia and procedural guidance for these anatomically complex patients.
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