Ebstein’s Anomaly of the Tricuspid Valve: an Overview of Pathology and Management

• Published in: Current cardiology reports 2020-10, Vol.22 (12), p.157-157, Article 157
• Main Authors: Possner, Mathias, Gensini, Francisco J., Mauchley, David C., Krieger, Eric V., Steinberg, Zachary L.
• Format: Article
• Language: English
• Subjects: Adult; Arrhythmias, Cardiac - etiology; Cardiology; Ebstein Anomaly - diagnostic imaging; Ebstein Anomaly - surgery; Heart Defects, Congenital; Humans; Medicine; Medicine & Public Health; Section Editor; Topical Collection on Valvular Heart Disease; Tricuspid Valve - diagnostic imaging; Tricuspid Valve - surgery; Tricuspid Valve Insufficiency; Valvular Heart Disease (TL Kiefer
• ISSN: 1523-3782; 1534-3170
• DOI: 10.1007/s11886-020-01412-z

Purpose of Review Ebstein’s anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population. Recent Findings Increased understanding of anatomic accessory atrioventricular pathways in EA has resulted in an improvement in ablation techniques and long-term freedom of atrial arrhythmia recurrence. Summary Despite an improvement in understanding and recognition of EA, significant disease heterogeneity and complex treatment options continue to challenge providers, with the best outcomes achieved at expert congenital heart disease centers.