The 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.
Thirty 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.
Anatomy: 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.
The 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.
The characteristics of Ebstein™s anomaly of the tricuspid valve include tethering of the leaflets to the ventricular wall and redundancy and dysplasiaof 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 1-6. 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 5-7. 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. Moreover, in the infundibular portion of the ventricular outflow tract, myocardial fibers are diminished in number, making it very thin. Subpulmonary and pulmonary obstructions can also be present 6,7. In addition, 25% of patients with Ebstein™s anomaly also present with a Wolf-Parkinson-White pre-excitation syndrome 8.
The hemodynamic effects are related to the tricuspid regurgitation, the existence of an atrial septal defect and the degree of dysfunction of the atrialized portion of the right ventricle. Some cases have tricuspid stenosis owing to reduction of the functional valve opening secondary to fusion of the leaflets. In such patients an atrial septal defect is obligatory for survival 8./>/>/>
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