Morphological Aspects of Ebstein's Anomaly in Adults

Abstract

The essence of the Ebstein's malformation is that the tricuspid valve leaflets do not attach normally to the valve annulus, and the effective orifice is displaced downward into the right ventricular cavity at the junction of the inlet and trabecular components of the right ventricle. Only the septal and posterior leaflets are displaced and divide the right ventricle into two portions. The inlet portion is usually integrated functionally with the right atrium ("atrialized portion"), while the other, including the trabecular and outlet portions, constitutes the functional right ventricle. The proximal atrialized right ventricle often has a wall thinner than the distal functional right ventricle, due to partial congenital absence of myocardium. An atrial septal defect is present in more than one-third of hearts, and the majority of the remainder has a patent foramen ovale resulting in a right-to-left shunt. The downward displacement of the septal tricuspid valve leaflet is associated with discontinuity of the central fibrous body and septal atrioventricular ring, thus creating a potential substrate for accessory atrioventricular connections and ventricular pre-excitation making the patient at risk of sudden death. Angiography has demonstrated that a significant number of patients with Ebstein's anomaly also have morphofunctional abnormalities of the left ventricle, which may be explained by increased fibrosis in the left ventricular wall and ventricular septum as demonstrated by histological studies. Regarding embryology, the leaflets and tensile apparatus of the tricuspid valve are believed to be formed mostly by a process of delamination of the inner layers of the inlet zone of the right ventricle. The downward displacement of the leaflets in Ebstein's anomaly suggests that delamination from the inlet portion failed to occur.

Surgical Management of Ebstein’s Anomaly with Atrial Septal Defect in a Young Lady: A Case Report

“…One third present with an ASD and majority of the reminder as PFO. 8 Our patient also presented with similar findings. EA is a rare disease but due to recent advancement of diagnostic facilities, cases are diagnosed and reported at a higher incidence.…”

The Normal Offset of the Tricuspid Septal Leaflet in the Fetus

“…Lack of the typical apical displacement of the tricuspid valve may indicate the presence of an AV septal defect, whereas exaggerated displacement of the tricuspid valve may be indicative of Ebstein anomaly. 1,2,[12][13][14] Either finding can have considerable clinical importance.…”

“…10,17 Previous studies have evaluated various ratios such as the cardiothoracic ratio, right and left atrial heights, valve annulus diameters, and others. 9,12,[16][17][18][19][20] The quantitative evaluation of mitral and tricuspid valve displacement by echocardiography has been studied in the pediatric and adult populations. 9,10 Studies in adult and pediatric groups have also correlated the degree of apical displacement or absence of the septal leaflet of the tricuspid valve with clinical outcome.…”

Ebstein’s Anomaly

“…[2][3][4][5][6] This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, "Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation." 7,8 The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly.…”