Abstract
ObjectiveThe aim of this investigation is to demonstrate that in Ebstein's Anomaly (EA) the right ventricle (RV) is affected in its three portions and to establish an anatomoechocardiographic correlation between the anatomic features and the equivalent echocardiographic images.MethodsThirty hearts with EA were studied. The alterations of each portions of the RV were described. Fifty adult patients with this anomaly were studied by echocardiography.ResultsAnatomy: All hearts had atrial situs solitus, 27 had concordant atrioventricular connection and 3 discordant, of these 2 had transposition of the great arteries (TGA) and one double outlet right ventricle (DORV). The degree of tricuspid valve (TV) displacement showed a spectrum from I to III. The inlet of the RV was markedly thin in 27. The trabecular portion had multiples muscular bands in all. The outlet portion was dilated in 20 and stenotic in 5. In 25 atrial septal defects were found. Echocardiography: All patients had atrial situs solitus, 42 with concordant atrioventricular connection and 8 with discordant, of these last patients 5 had TGA and 3 DORV. The degree of TV displacement varied from I to III. The inlet of RV was markedly thin in 42. The trabecular portion had muscular bands in 45. The outlet portion was dilated in 31 and stenotic in 11. In 30 atrial septal defects were found.ConclusionThe EA affects the whole RV and the anatomoechocardiographic correlation provides an appropriate understanding of echocardiographic images in terms of a precise diagnosis, therapeutic decisions and prognosis.
Highlights
The characteristics of Ebstein's anomaly of the tricuspid valve include tethering of the leaflets to the ventricular wall and redundancy and dysplasia of the leaflets
The apical trabecular portion of the right ventricular free wall shows a characteristic pattern of anomalous muscular bands that connect the ventricular septum to the free wall [6]
Our study provides significant information based on an analysis of the three portions of the right ventricle and the anatomo-echocardiographic correlation in Ebstein's anomaly
Summary
The characteristics of Ebstein's anomaly of the tricuspid valve include tethering of the leaflets to the ventricular wall and redundancy and dysplasia of the leaflets. The variable degrees of redundancy and dysplasia determine the displacement of the functional tricuspid opening toward the trabecular portion and outflow tract of the right ventricle [1,2,3,4,5,6]. These alterations of the tricuspid valve have traditionally constituted the most conspicuous aspects of this congenital cardiac malformation [1,4,5]. The histopathology of the right ventricular wall reveals a decrease or total absence of myocardial fibers in the inlet portion [57].
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