![]() ![]() More than one-half of Ebstein’s patients will also have some degree of cyanosis from an interatrial communication (either a sprung foramen ovale or atrial septal defect), which allows right-to-left shunting. This in turn causes right atrial (RA) enlargement with expansion of the true right atrioventricular (AV) groove, as well as dilation of the “atrialized” portion of the RV above the functional TV orifice, where the tissue is remarkably thin-walled. As a consequence, the functional TV orifice becomes distorted and displaced down into the apical portion of RV, which results in variable degrees of regurgitation and stenosis ( Figure 1). The anterior leaflet tends to retain mobility but is typically elongated, fenestrated, and tethered. The central anatomic derangement involves a distinctive malformation of the tricuspid valve (TV) with adherence of the septal and posterior leaflets to the underlying right ventricular (RV) myocardium due to failure of embryological delamination (1). This review will explore the gross and microscopic anatomy of Ebstein’s anomaly with attention to features that could be important to both arrhythmogenesis and ablation therapy in this unique population.Įbstein’s anomaly is a relatively rare congenital cardiac malformation that can pose serious management challenges from both a hemodynamic and electrophysiological perspective. Furthermore, for reasons that are only now being fully appreciated, successful catheter ablation of APs in this setting is frequently more challenging than would be expected in a structurally normal heart. Although it is logical to postulate a link between the anatomic deformity and the conduction abnormality, the exact nature of this link is still not fully understood and remains a fertile area for investigation that might shed light on abnormal conduction pathways in many other forms of heart disease. It is the only congenital heart defect with such a dramatic predisposition toward APs. In addition to the hemodynamic burden of the actual valve defect, Ebstein’s patients must also contend with an extraordinarily high incidence of tachyarrhythmias, most of which can be attributed to accessory atrioventricular pathways (APs) located along the posterior and septal border of the tricuspid valve where the valve leaflets are most abnormal. Ebstein’s anomaly of the tricuspid valve is a relatively rare form of congenital heart disease that has long been a challenge to electrophysiologists and cardiac surgeons. ![]()
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