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Ebstein's anomaly of the tricuspid valve

Authors
Brojendra N Agarwala, MD
Ziyad M Hijazi, MD, MPH, FAAP, FACC, MSCAI, FAHA
Joseph Dearani, MD
Section Editor
Heidi M Connolly, MD, FASE
Deputy Editor
Susan B Yeon, MD, JD, FACC

INTRODUCTION

Ebstein's anomaly is a congenital malformation that is characterized primarily by abnormalities of the tricuspid valve and right ventricle (figure 1). The estimated risk of Ebstein’s anomaly in the general population is 1 in 20,000 live births with no predilection for either gender [1-3]. For unknown reasons, it occurs with higher frequency in infants of mothers who take lithium during early pregnancy [4,5]. (See "Teratogenicity, pregnancy complications, and postnatal risks of antipsychotics, benzodiazepines, lithium, and electroconvulsive therapy", section on 'Lithium'.)

MORPHOLOGY

Tricuspid valve — The morphology of the tricuspid valve in Ebstein's anomaly, and consequently the clinical presentation, is highly variable. The tricuspid valve leaflets are malformed and partly attached to the fibrous tricuspid valve annulus and partly to the right ventricular endocardium (image 1).

The anterior leaflet is the largest leaflet and is usually attached to the tricuspid valve annulus.

The posterior and septal leaflets are vestigial or absent as there is failure of delamination (separation of the layer of valve tissue) from the myocardium. When present, the free edges are generally displaced posteriorly and downward from the atrioventricular junction and progressively toward the right ventricular outflow tract and apex.

The leaflets of the tricuspid valve may have free leading edges with chordae, or be directly attached to papillary muscle(s) or the underlying myocardium. The tricuspid valve is funnel-shaped ("sail-like"), incompetent, and rarely stenotic.

                       

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Literature review current through: Nov 2016. | This topic last updated: Fri Sep 18 00:00:00 GMT+00:00 2015.
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