Ebstein's anomaly: Glimpses in pathophysiology, prevalence, diagnostic updates, and treatment strategies through evolution

Ebstein's anomaly is a rare congenital heart disease in which the leaflets of the tricuspid valve are displaced apically and culminate in an atrialized right ventricle, tricuspid regurgitation, and right-sided heart dysfunction. This anomaly provides critical clinical variability from neonatal cyanosis to adult arrhythmias and is hence vulnerable to diverse management protocols. This review summarizes Ebstein's anomaly in terms of its pathophysiology, prevalence, diagnostic advances, and treatment, highlighting lacunas and directions for future investigations. This was a systematic literature review from 2000--2023 in the PubMed, Scopus, and Web of Science databases. The search terms used were "Ebstein's anomaly," "tricuspid valve malformation," and "cone reconstruction surgery." Preferred studies included imaging, surgical advancement, and the management of arrhythmia. The historical and recent clinical findings also provide some key references. Ebstein's anomaly shows a wide range of clinical spectra. Advances in diagnostic imaging have enhanced the understanding and planning of surgery via 3D echocardiography and cardiac magnetic resonance imaging (MRI). Cone reconstruction surgery has become the standard of care for repairing the tricuspid valve and has improved outcomes, especially in pediatric patients. Neonates are still a challenge and most often require staged interventions or ECMO support. Most arrhythmias, including Wolff–Parkinson–White syndrome, require a combination of antiarrhythmic therapy, catheter ablation, and surgical interventions. Challenges remain in optimizing care for neonates and managing long-term complications. There have been considerable advancements in the management and results of Ebstein's anomaly. Further research must be conducted on genetic factors, new therapies, and less invasive techniques to solve ongoing problems.